Fostamatinib effectiveness and safety for immune thrombocytopenia in clinical practice.

Fostamatinib, a recently approved syk inhibitor used in adult primary immune thrombocytopenia (ITP), has been shown to be safe and effective in this disorder. However, clinical trial results may not be similarly reproduced in clinical practice. Here 138 ITP patients (both primary and secondary) from 42 Spanish centers who had been treated with fostamatinib were evaluated prospectively and retrospectively. The median age of our cohort (55.8% women) was 66 years (interquartile range, IQR, 56-80 years). The median time since ITP diagnosis at fostamatinib initiation was 51 months (IQR, 10-166 months). The median number of therapies prior to fostamatinib initiation was 4 (IQR, 2-5), including eltrombopag (76.1%), romiplostim (57.2%) and intravenous immunoglobulins (IVIG) (44.2%). Fifty-eight patients (42.0%) had signs/symptoms of bleeding in the month prior to treatment initiation. 79.0% of patients responded to fostamatinib with 53.6% complete responses (platelet count > 100 x 109 /L). Eighty-three patients (60.1%) received fostamatinib monotherapy achieving a high response rate (85.4%). The proportion of time in response during the 27-month period examined was 83.3%. The median time to platelet response was 11 days (IQR, 7-21 days). Sixty-seven patients (48.5%) experienced adverse events, mainly grade 1-2, the commonest of which were diarrhea (n = 28) and hypertension (n = 21). One patient had deep venous thrombosis and one patient developed acute myocardial infarction. Fostamatinib was shown to be effective with good safety profile in patients with primary and secondary ITP across a wide age spectrum in this real-world study.

Treatment with fostamatinib in patients with immune thrombocytopenia: Experience from the Andalusian region in Spain-The Fostasur Study.

Immune thrombocytopenia (ITP) is characterized by low platelet counts (PLTs) and an increased risk of bleeding. Fostamatinib, a spleen tyrosine kinase inhibitor, has been approved as a second-line treatment for ITP. Real-world data on fostamatinib are lacking. This observational, retrospective, multicentre study, conducted in the Andalusia region of Spain, evaluated 44 adult primary ITP patients (47.7% female; median age 58 years; newly diagnosed ITP 6.8%; persistent 13.6%; chronic 79.5%; median four prior treatments) after ≥ 4 weeks of fostamatinib therapy. The median PLT at the initiation of fostamatinib was 15 × 109/L. Common reasons for starting fostamatinib were refractoriness or intolerance to prior therapy, oral medication preference, history of thrombosis and cardiovascular risk. Dosing was individualized based on efficacy and tolerance. After 2 weeks, global response rate was 56.8% (response and complete response). Response rates were 70.5%, 62.5% and 64% at 4 weeks, 12 weeks and at the end of the study respectively. Adverse events were mild, and no patients discontinued as a result. This real-world study demonstrated a response rate similar to fostamatinib as seen in the pivotal clinical trials while including newly diagnosed patients and allowing for individualized dosing.

"To be, or not be satisfied in NPOs": a serial multiple mediation and clustering analysis of paid staff and volunteers' profiles.

Introduction: Non-profit organizations (NPOs) are a complex working context whose main characteristic resides in the dichotomy between paid staff and volunteers. Despite its benefits for goal achievement, this circumstance can be also a challenge, for both groups' interaction, for their comprehension of their own role and to HR management. The aim of this study was to explore factors that may contribute to promote job satisfaction among NPOs' members. Methods: Combining two different data analyses, serial multiple mediation analyses and cluster analyses, first we analyze whether there are differences between paid staff and volunteers in perceived intrapersonal conflict and performance and its effect on job satisfaction, and second, we analyze whether there is an additional profiles distribution that reflects more adequately the reality of NPOs, despite the formal roles that coexist in these organizations (paid staff and volunteers). Results: Results confirm that paid staff and volunteers differ on their perceived job satisfaction level, with volunteers being more satisfied. This relationship is serially mediated by role conflict, role ambiguity, and performance. Another characteristic of the NPOs is that the dichotomy between paid staff and volunteers does not capture well the reality of the labor relations between members of both groups and the organization. To explore this phenomenon, we perform a cluster analysis based on paid staff and volunteers' perceptions. Cluster analyses demonstrate the existence of three rather homogenous profiles. Discussion: Additionally, practical implications for HR management in NPOs and future research lines to understand this organizational context dynamics are also discussed.

¿Es recomendable el uso de PAMORAS en paciente en cuidados paliativos? / Is it advisable to use PAMORAS for patients in palliative care?

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Dilema en la toma de decisiones: paciente con cáncer avanzado y esquizofrenia / Decision-making dilemma: a patient with advanced cancer and schizophrenia

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Inside "Pandora's Box" of Solidarity: Conflicts Between Paid Staff and Volunteers in the Non-profit Sector.

Non-profit organizations (NPOs) are quite complex in terms of organizational structure, diversity at the workplace, as well as motivational mechanisms and value rationality. Nevertheless, from the perspective of organizational psychology, the systematic analysis of this context is scarce in the literature, particularly regarding conflicts. This qualitative study analyzes types, prevalence, and consequences of conflicts in a large NPO considering as theoretical framework several consolidated organizational psychology theories: conflict theory, social comparison theory, and equity theory. Conflicts were analyzed taking into account volunteers' perspective, who have been the consistent protagonist in NPO research, but also considering paid staff's perspective as one of the main stakeholders in these organizations, whose relative power has increased in the past decade due to the professionalization of the NPO's sector. Results confirmed the existence of four types of conflicts: task, process, status, and relationship conflicts. Relationship conflict is the least reported type, revealing the protection factor that values and engagement with a social aim have on this organizational context. The most relevant finding is the strong difference between paid staff and volunteers in conflict perceptions, showing paid staff, overall, higher levels of conflicts than volunteers. Findings also show stronger negative consequences for paid staff compared to volunteers. Theoretical and practical implications are discussed.

Tramadol: la ruleta analgésica / Tramadol: analgesic roulette

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Approaching the Discriminatory Work Environment as Stressor: The Protective Role of Job Satisfaction on Health.

Discrimination is a complex phenomenon with adverse consequences at personal and organizational levels. Past studies have demonstrated that workers who are victims of discrimination might show less job satisfaction, less organizational commitment and worse levels of health and productivity. Although most research has focused on the effects of discrimination on victims, less is known about the extent to which discrimination produces consequences on workers who perceive the existence of a discriminatory work environment. The goal of this article is to analyze the consequences of the perception of a discriminatory work environment on employees' health. The importance of this relationship is studied taking into account the mediating effect of job satisfaction. In order to reach this goal a cross-sectional study was carried out with a sample of 1633 Italian workers (male = 826, female = 764), employed in private and public sectors, and in different hierarchical positions. Results suggest that the perception of a discriminatory work environment is negatively associated with employees' health. This relationship is partially mediated by job satisfaction (R (2) = 0.17). This study demonstrates that perceiving a discriminatory work environment might have a negative impact on workers' health. A higher level of job satisfaction might buffer this effect. These findings have several practical implications. On the one hand, Human Resource Managers need to intervene in order to recognize and diminish implicit biases, creating a healthy and inclusive environment (e.g., through training, diversity policies, etc.). On the other hand, promoting job satisfaction (e.g., providing mechanisms of voice) might help workers to preserve their well-being, coping with the negative effects of a discriminatory work environment.

Congenital lupus with multiorgan involvement: a case report and review of literature.

Neonatal lupus erythematosus (NLE) is an autoimmune disease caused by transplacental antibodies that can damage fetal tissue and cause various findings. With the exception of congenital heart block, which can be easily recognized at birth because of neonatal cardiac monitoring during the delivery and immediately after birth, most cases of NLE are recognized within days to weeks of life, but fewer than 10 cases with findings present at birth have been reported. We report the case of a newborn with extensive cutaneous eruption at the time of birth and multisystemic involvement, including hematologic, respiratory, hepatic, and neurologic involvement.

Eculizumab in dense-deposit disease after renal transplantation.

BACKGROUND: Dense-deposit disease (DDD) is a rare glomerulopathy characterized by electron-dense deposits in the glomerular basement membrane. About 50 % of patients with DDD progress to end-stage kidney disease and require dialysis within 10 years of diagnosis, and the disease often recurs after renal transplantation. CASE-DIAGNOSIS/TREATMENT: We describe a 14-year-old girl with recurrent DDD in her transplanted kidney. Clinical onset was at 8 years of age, when steroid-resistant nephrotic syndrome was diagnosed with microhematuria, severe hypocomplementemia and normal kidney function. Although remission was initially observed after several plasma exchanges, nephrotic proteinuria returned and kidney function further declined 1 year later. The patient received a living-related kidney transplant. Initial allograft function was good, but proteinuria reappeared 3 months after transplantation, accompanied by a slight deterioration in kidney function. After histological confirmation of DDD recurrence and subsequent management with plasmapheresis, the patient was treated for 30 months with eculizumab, a humanized monoclonal antibody that binds to C5 complement protein. This intervention proved effective and resulted in complement inhibition, sustained remission of proteinuria and preservation of renal function. A graft biopsy 6 months later showed no progression of the renal lesions. CONCLUSIONS: Early clinical and histological recurrence of DDD in the transplanted kidney in this 14-year-old patient was treated for 30 months with eculizumab. The patient remains asymptomatic, has no proteinuria and her kidney function is intact.

Lupus eritematoso neonatal: revisión de casos en los últimos 5 años / Neonatal lupus erythematosis: A five-year case review

El lupus eritematoso neonatal es una enfermedad rara del recién nacido producida por el paso transplacentario de autoanticuerpos maternos. Las 2 formas de presentación más frecuentes son la dermatológica (lupus eritematoso subagudo) y el bloqueo auriculoventricular completo (BAVC). También puede producir afectación hematológica, hepática, neurológica, respiratoria y digestiva. Presentamos una revisión de 4 casos diagnosticados en los últimos 5 a˜nos en nuestra Unidad de Neonatología, que reflejan el amplio espectro clínico con el que se puede presentar esta enfermedad (un caso de BAVC, uno con afectación multisistémica y 2 casos con expresión cutánea), los diferentes patrones de autoanticuerpos (con un predominio de anticuerpos anti-SSA), la desaparición de autoanticuerpos en todos los casos antes del año de edad y la posibilidad de aparición de colagenopatías en el futuro, como ocurrió en uno de nuestros casos (AU)

Neonatal lupus erythematosus is an infrequent disease seen in newborns. It is caused by transplacental maternal autoantibody passage. Cutaneous involvement and congenital heart block (CHB) are the most common affections, although it may involve multiple organs like the liver, lungs, blood, nervous or digestive systems. This article present a review of the four cases diagnosed in the past five years in a Neonatal Unit, which shows the different clinical spectrum which can develop around this disease (CHB, multisystemic affection and two cutaneous cases), different autoantibodies (specially anti-SSA) with an early negativization during the first year of life and the possibility of future collagen vascular disease as occurred in one case (AU)

Association of C4d deposition with clinical outcomes in IgA nephropathy.

BACKGROUND AND OBJECTIVES: Several studies have suggested that activation of the complement system is a contributing pathogenic mechanism in IgA nephropathy (IgAN). C4d staining is an inexpensive and easy-to-perform method for the analysis of renal biopsies. This study aimed to assess the clinical and prognostic implications of C4d staining in IgAN. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective cohort study included 283 patients with IgAN in 11 hospitals in Spain who underwent a renal biopsy between 1979 and 2010. The primary predictor was mesangial C4d staining. Secondary predictors included demographic, clinical, and laboratory characteristics, and Oxford pathologic classification criteria. The primary end point was the cumulative percentage of patients who developed ESRD, defined as onset of chronic dialysis or renal transplantation. C4d was analyzed by immunohistochemical staining using a polyclonal antibody. Kaplan-Meier and Cox proportional hazards analyses were performed to evaluate the effect of C4d staining on renal survival. RESULTS: There were 109 patients (38.5%) and 174 patients (61.5%) who were classified as C4d positive and C4d negative, respectively. Renal survival at 20 years was 28% in C4d-positive patients versus 85% in C4d-negative patients (P<0.001). Independent risk factors associated with ESRD were as follows: proteinuria (hazard ratio [HR] per every 1 g/d increase. 1.16; 95% confidence interval [95% CI], 1.03 to 1.31; P=0.01), eGFR (HR per every 1 ml/min per 1.73 m(2) increase, 0.96; 95% CI, 0.94 to 0.97; P<0.001), T2 Oxford classification (tubular atrophy/interstitial fibrosis, >50%; HR, 4.42; 95% CI, 1.40 to 13.88; P=0.01), and C4d-positive staining (HR, 2.45; 95% CI, 1.30 to 4.64; P=0.01). CONCLUSIONS: C4d-positive staining is an independent risk factor for the development of ESRD in IgAN. This finding is consistent with the possibility that complement activation is involved in the pathogenesis of this disease.

Neonatal lupus erythematosis: a five-year case review.

Neonatal lupus erythematosus is an infrequent disease seen in newborns. It is caused by transplacental maternal autoantibody passage. Cutaneous involvement and congenital heart block (CHB) are the most common affections, although it may involve multiple organs like the liver, lungs, blood, nervous or digestive systems. This article present a review of the four cases diagnosed in the past five years in a Neonatal Unit, which shows the different clinical spectrum which can develop around this disease (CHB, multisystemic affection and two cutaneous cases), different autoantibodies (specially anti-SSA) with an early negativization during the first year of life and the possibility of future collagen vascular disease as occurred in one case.

Clofazimine as elective treatment for granulomatous cheilitis.

Cheilitis granulomatosa (CG) is a rare, idiopathic inflammatory disorder that usually affects young adults and clinically is characterized by diffuse, non-tender, soft to firm swelling of one or both lips. A variant of granulomatous cheilitis is Melkersson-Rosenthal syndrome when associated with facial paralysis and furrowed tongue. Several treatments have been used with variable success. We report 3 cases of GC treated with oral clofazimine 100 to 200 mg daily for 3 to 6 months obtaining regression of lesions in all treated cases. Hyperpigmentation and elevation of liver enzymes were observed as side effects.

Granuloma anular de disposición lineal asociado a neoplasia esofágica / Linear granuloma annulare associating esophageal neoplasia

Entre las múltiples etiologías implicadas en el granuloma anular existen casos descritos asociados a tumores malignos sobre todo de estirpe hematológica. En estos pacientes no es infrecuente la presentación atípica de esta dermatosis. Describimos el caso de un varón de 67 años diagnosticado de adenocarcinoma mucosecretor de esófago que desarrolló una placa violácea de disposición lineal en la región lateral del cuello, durante el transcurso de su enfermedad. El estudio histopatológico descartó la presencia de células neoplásicas, observándose hallazgos compatibles con granuloma anular. Se discute la posible relación de esta dermopatía con neoplasias malignas y se destaca la inusual forma de presentación clínica (AU)