Acquired vibratory angioedema with a severe impact on work performance.

Vibratory urticaria/angioedema is an inducible type of urticaria that may be elicited by vibratory stimuli from work tool and instruments. A 39-year-old male construction worker was diagnosed with acquired vibratory angioedema associated with chronic spontaneous urticaria involving a severe work performance impairment. Response to omalizumab was immediate and complete. We evaluated the Urticarial Activity Score; the response to the vibratory angioedema provocation vortex test; and evolution of analytical parameters (e.g. high-affinity IgE receptor expression on peripheral blood basophils, C-reactive protein, D-dimer and IgE levels). Vibratory angioedema may be misdiagnosed as delayed pressure urticaria if the appropriate specific provocation tests are not performed. According to our knowledge, this is the first time a pneumatic hammer is described as a trigger; the first case responding to omalizumab; and the second published case associated with spontaneous chronic urticaria.

Spanish and Portuguese Societies of Internal Medicine consensus guideline about best practice in end-of-life care.

AIM: To develop consensus recommendations about good clinical practice rules for caring end-of-life patients. METHODS: A steering committee of 12 Spanish and Portuguese experts proposed 37 recommendations. A two rounds Delphi method was performed, with participation of 105 panelists including internists, other clinicians, nurses, patients, lawyers, bioethicians, health managers, politicians and journalists. We sent a questionnaire with 5 Likert-type answers for each recommendation. Strong consensus was defined when >95% answers were completely agree or >90% were agree or completely agree; and weak consensus when >90% answers were completely agree or >80% were agree or completely agree. RESULTS: The panel addressed 7 specific areas for 37 recommendations spanning: identification of patients; knowledge of the disease, values and preferences of the patient; information; patient's needs; support and care; palliative sedation, and after death care. CONCLUSIONS: The panel formulated and provided the rationale for recommendations on good clinical practice rules for caring end-of-life patients.

Guía de práctica clínica de consenso sobre buenas prácticas en los cuidados al final de la vida de las Sociedades Española y Portuguesa de Medicina Interna / Spanish and Portuguese Societies of Internal Medicine consensus guideline about best practice in end-of-life care

Objetivo Establecer recomendaciones de consenso sobre normas de buena práctica clínica en la atención a los pacientes al final de la vida. Métodos Un comité de 12 expertos españoles y portugueses propuso 37 recomendaciones. Se realizó un Proceso Delphi a dos rondas, con participación de 105 panelistas incluyendo internistas, otros médicos clínicos, enfermeras, enfermos, juristas, expertos en bioética, gestores sanitarios, políticos y periodistas. Para cada recomendación se envió un cuestionario con cinco respuestas tipo Likert. Se definió consenso fuerte cuando > 95% de las respuestas estaban totalmente de acuerdo o > 90% estaban de acuerdo y totalmente de acuerdo; consenso débil cuando > 90% estaban totalmente de acuerdo o > 80% estaban de acuerdo y totalmente de acuerdo. Resultados El panel abordó siete áreas específicas con 37 recomendaciones que abarcaban: Identificación de los pacientes; Conocimiento, valores y preferencias del paciente; Información; Necesidades del paciente; Atención y cuidados; Sedación paliativa y Atención tras la muerte. Conclusiones Un Proceso Delphi con participación multidisciplinar ha permitido establecer normas de buena práctica clínica en la atención al final de la vida con consenso de enfermos, agentes sociales y profesionales sanitarios (AU)

Aim To develop consensus recommendations about good clinical practice rules for caring end-of-life patients. Methods A steering committee of 12 Spanish and Portuguese experts proposed 37 recommendations. A two rounds Delphi method was performed, with participation of 105 panelists including internists, other clinicians, nurses, patients, lawyers, bioethicians, health managers, politicians and journalists. We sent a questionnaire with 5 Likert-type answers for each recommendation. Strong consensus was defined when > 95% answers were completely agree or > 90% were agree or completely agree; and weak consensus when > 90% answers were completely agree or > 80% were agree or completely agree. Results The panel addressed 7 specific areas for 37 recommendations spanning: identification of patients; knowledge of the disease, values and preferences of the patient; information; patient's needs; support and care; palliative sedation, and after death care Conclusions The panel formulated and provided the rationale for recommendations on good clinical practice rules for caring end-of-life patients (AU)

Spanish and Portuguese Societies of Internal Medicine consensus guideline about best practice in end-of-life care. / Guía de práctica clínica de consenso sobre buenas prácticas en los cuidados al final de la vida de las Sociedades Española y Portuguesa de Medicina Interna.

AIM: To develop consensus recommendations about good clinical practice rules for caring end-of-life patients. METHODS: A steering committee of 12 Spanish and Portuguese experts proposed 37 recommendations. A two rounds Delphi method was performed, with participation of 105 panelists including internists, other clinicians, nurses, patients, lawyers, bioethicians, health managers, politicians and journalists. We sent a questionnaire with 5 Likert-type answers for each recommendation. Strong consensus was defined when > 95% answers were completely agree or > 90% were agree or completely agree; and weak consensus when > 90% answers were completely agree or > 80% were agree or completely agree. RESULTS: The panel addressed 7 specific areas for 37 recommendations spanning: identification of patients; knowledge of the disease, values and preferences of the patient; information; patient's needs; support and care; palliative sedation, and after death care. CONCLUSIONS: The panel formulated and provided the rationale for recommendations on good clinical practice rules for caring end-of-life patients.

End-of-life care for patients hospitalised in internal medicine departments. / Cuidados en los últimos días de vida en los pacientes hospitalizados en medicina interna.

OBJECTIVES: To describe the care provided at the end of life for patients who die in internal medicine departments. METHODS: An observational, cross-sectional, retrospective multicentre, clinical audit study was conducted where each hospital included the first 10 patients who died in the internal medicine department starting on December 1, 2015. We collected demographic and clinical data and information regarding the circumstances and care at the time of death. RESULTS: The study included 1,447 patients with a median age of 84 years. Of these, 1,065 (74.3%) were polypathological, 751 (51.9%) were terminal and 248 (17.1%) had cancer. For the terminal patients, do-not-resuscitate orders were established for 539 (73.3%), and palliative sedation was performed for 422 (57.4%). There was no record as to whether psychological, religious or grief care was provided in 32%, 64.8% and 44.1% of the terminal patients, respectively. The patients with cancer were more often competent to make decisions (54.4% vs. 15.5%; P<.001), knew their prognosis (42.6% vs. 8.6%; P<.001), received psychological care (24.9% vs. 8.6%; P<.001), died in an individual room (64.6% vs. 44.4%; P<.001) and were accompanied (81.9% vs. 71.9%; P=.003). Their relatives also more frequently received grief care (15.6% vs. 8.2%; P=.002). CONCLUSIONS: There is insufficient recording in the medical history as to the end-of-life care. There are differences in the care provided to patients with cancer and to those without cancer.

Characterization of CD34+ hematopoietic cells in systemic mastocytosis: Potential role in disease dissemination.

BACKGROUND: Recent studies show that most systemic mastocytosis (SM) patients, including indolent SM (ISM) with (ISMs+) and without skin lesions (ISMs-), carry the KIT D816V mutation in PB leukocytes. We investigated the potential association between the degree of involvement of BM hematopoiesis by the KIT D816V mutation and the distribution of different maturation-associated compartments of bone marrow (BM) and peripheral blood (PB) CD34+ hematopoietic precursors (HPC) in ISM and identified the specific PB cell compartments that carry this mutation. METHODS: The distribution of different maturation-associated subsets of BM and PB CD34+ HPC from 64 newly diagnosed (KIT-mutated) ISM patients and 14 healthy controls was analyzed by flow cytometry. In 18 patients, distinct FACS-purified PB cell compartments were also investigated for the KIT mutation. RESULTS: ISM patients showed higher percentages of both BM and PB MC-committed CD34+ HPC vs controls, particularly among ISM cases with MC-restricted KIT mutation (ISMMC ); this was associated with progressive blockade of maturation of CD34+ HPC to the neutrophil lineage from ISMMC to multilineage KIT-mutated cases (ISMML ). Regarding the frequency of KIT-mutated cases and cell populations in PB, variable patterns were observed, the percentage of KIT-mutated PB CD34+ HPC, eosinophils, neutrophils, monocytes and T cells increasing from ISMs-MC and ISMs+MC to ISMML patients. CONCLUSION: The presence of the KIT D816V mutation in PB of ISM patients is associated with (early) involvement of circulating CD34+ HPC and multiple myeloid cell subpopulations, KIT-mutated PB CD34+ HPC potentially contributing to early dissemination of the disease.

[Reason for consultation: Weight loss]. / Motivo de consulta: pérdida de peso.

BACKGROUND AND OBJECTIVE: Unintentional weight loss is frequent reason to visit a doctor and it has multiple diagnostic possibilities. The objective of this study is to examine the background of the patients who seek consultation for weight loss and to establish the relationship between weight loss and neoplasia. METHOD: An analysis was performed on the demographic data, quantified weight loss, accompanying symptomatology, and diagnosis of patients who sought medical advice for unintentional weight loss during the year 2015. RESULTS: A total of 226 patients were included, of whom 44.2% of them had an intentional weight loss ≥ 5% in 6 months. The most frequent diagnosis in this group was a neoplasia, whereas in the rest of patients the most common diagnosis was a gastrointestinal disease. In light of this study we can conclude that there is a relationship between unintentional weight loss≥5% and the presence of neoplasia. CONCLUSION: An unintentional weight loss greater than 5% in the previous 6 months is associated with the presence of neoplastic diseases, and therefore requires further diagnostic study.

Increased IL6 plasma levels in indolent systemic mastocytosis patients are associated with high risk of disease progression.

Systemic mastocytosis (SM) is a heterogeneous disease with altered interleukin (IL)-6 and IL13 plasma levels. However, no study has simultaneously investigated the plasma levels of IL1ß, IL6, IL13, CCL23 and clusterin in SM at diagnosis and correlated them with disease outcome. Here we investigated IL1ß, IL6, IL13, CCL23 and clusterin plasma levels in 75 SM patients--66 indolent SM (ISM) and 9 aggressive SM--and analyzed their prognostic impact among ISM cases grouped according to the extent of hematopoietic involvement of the bone marrow cells by the KIT D816V mutation. Although increased IL1ß, IL6 and CCL23 levels were detected in SM patients versus healthy controls, only IL6 and CCL23 levels gradually increased with disease severity. Moreover, increased IL6 plasma levels were associated with ISM progression to more aggressive disease, in particular among ISM patients with multilineal KIT mutation (ISM-ML), these patients also showing a higher frequency of organomegalies, versus other ISM-ML patients. Of note, all ISM patients who progressed had increased IL6 plasma levels already at diagnosis. Our results indicate that SM patients display an altered plasma cytokine profile already at diagnosis, increased IL6 plasma levels emerging as an early marker for disease progression among ISM cases, in particular among high-risk ISM patients who carry multilineage KIT mutation.

[Identifying gaps between guidelines and clinical practice in Clostridium difficile infection]. / Diferencias entre las recomendaciones y la práctica clínica en la infección por Clostridium difficile.

OBJECTIVES: The first aim was to determine whether patients are being treated in accordance with the Society for Healthcare Epidemiology of America and the Infectious Diseases Society of America (IDSA/SHEA) Clostridium difficile guidelines and whether adherence impacts patient outcomes. The second aim was to identify specific action items in the guidelines that are not being translated into clinical practice, for their subsequent implementation. MATERIAL AND METHODS: A retrospective, descriptive study was conducted over a 36 month period, on patients with compatible clinical symptoms and positive test for C. difficile toxins A and/or B in stool samples, in an internal medicine department of a tertiary medical centre. Patient demographic and clinical data (outcomes, comorbidity, risk factors) and compliance with guidelines, were examined RESULTS: A total of 77 patients with C. difficile infection were identified (87 episodes). Stratified by disease severity criteria, 49.3% of patients were mild-moderate, 35.1% severe, and 15.6% severe-complicated. Full adherence with the guidelines was observed in only 40.2% of patients, and was significantly better for mild-moderate (71.0%), than in severe (7.4%) or severe-complicated patients (16.6%) (P<.003). Adherence was significantly associated with clinical cure (57% vs 42%), fewer recurrences (22.2% vs 77.7%), and mortality (25% vs 75%) (P<.01). The stratification of severity of the episode, and the adequacy of antibiotic to clinical severity, need improvement. CONCLUSIONS: Overall adherence with the guidelines for management of Clostridium difficile infection was poor, especially in severe and severe-complicated patients, being associated with worse clinical outcomes. Educational interventions aimed at improving guideline adherence are warranted.

Anaphylaxis as a clinical manifestation of clonal mast cell disorders.

Clonal mast cell disorders comprise a heterogeneous group of disorders characterized by the presence of gain of function KIT mutations and a constitutively altered activation-associated mast cell immunophenotype frequently associated with clinical manifestations related to the release of mast cells mediators. These disorders do not always fulfil the World Health Organization (WHO)-proposed criteria for mastocytosis, particularly when low-sensitive diagnostic approaches are performed. Anaphylaxis is a frequent presentation of clonal mast cell disorders, particularly in mastocytosis patients without typical skin lesions. The presence of cardiovascular symptoms, e.g., hypotension, occurring after a hymenoptera sting or spontaneously in the absence of cutaneous manifestations such as urticaria is characteristic and differs from the presentation of anaphylaxis in the general population without mastocytosis.