Angiosarcoma is a rare soft tissue sarcoma originating from endothelial cells. Given that current treatments for advanced disease have shown limited efficacy, alternative therapies need to be ...identified. In rare diseases, patient-derived cell models are crucial for screening anti-tumour activity. In this study, cell line models were characterised in 2D and 3D cultures. The cell lines’ growth, migration and invasion capabilities were explored, confirming them as useful tools for preclinical angiosarcoma studies. By screening a drug library, we identified potentially effective compounds: 8-amino adenosine impacted cell growth and inhibited migration and invasion at considerably low concentrations as a single agent. No synergistic effect was detected when combining with paclitaxel, gemcitabine or doxorubicin. These results suggest that this compound could be a potentially useful drug in the treatment of AGS.
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•2 and 3D cellular models of AGS constitute a promising in vitro drug screening tool.•Several compounds have been validated as effective in AGS cell lines.•8AA would be the most interesting drug with which to continue development in AGS.•8AA shows promising anti-tumour activity in vitro.•8AA demonstrated in vitro proliferation, migration and invasion inhibition.
We are presenting the case of a 53-year-old woman with a history of Sjögren syndrome and a secondary antiphospholipid syndrome admitted at the Nephrology department for the evaluation of renal ...failure. The patient was initially diagnosed with tubulointerstitial nephritis and subsequently a membranoproliferative type I glomerulonephritis, secondary to cryoglobulins during the course of the disease. Repeated renal biopsies were required to confirm the diagnosis.
In order to reduce the cardiovascular risk, morbidity and mortality of peritoneal dialysis (PD), a minimal level of small-solute clearances as well as a sodium and water balance are needed. The ...peritoneal dialysis solutions used in combination have reduced the complications and allow for a long-time function of the peritoneal membrane, and the preservation of residual renal function (RRF) in patients on peritoneal dialysis (PD) is crucial for the maintenance of life quality and long-term survival. This retrospective cohort study reviews our experience in automatic peritoneal dialysis (APD) patients, with end-stage renal disease (ESRD) secondary to diabetic nephropathy (DN) in comparison to non-diabetic nephropathy (NDN), using different PD solutions in combination.
Fifty-two patients, 29 diabetic and 23 non-diabetic, were included. The follow-up period was 24 months, thus serving as their own control.
The fraction of renal urea clearance (Kt) relative to distribution volume (V) (or total body water) (Kt/V), or creatinine clearance relative to the total Kt/V or creatinine clearance (CrCl) decreases according to loss of RRF. The loss of the slope of RRF is more pronounced in DN than in NDN patients, especially at baseline time interval to 12 months (loss of 0.29 mL/month vs. 0.13 mL/month, respectively), and is attenuated in the range from 12 to 24 months (loss of 0.13 mL/month vs. 0.09 mL/month, respectively). Diabetic patients also experienced a greater decrease in urine output compared to non-diabetic, starting from a higher baseline urine output. The net water balance was adequate in both groups during the follow up period. Regarding the balance sodium, no inter-group differences in sodium excretion over follow up period was observed. In addition, the removal of sodium in the urine output decreases with loss of renal function. The average concentration of glucose increase in the cycler in both groups (DN: baseline 1.44 ± 0.22, 12 months 1.63 ± 0.39, 24 months 1.73 ± 0.47; NDN: baseline 1.59 ± 0.40, 12 months 1.76 ± 0.47, 24 months 1.80 ± 0.46), in order to maintain the net water balance. The daytime dwell contribution, the fraction of day and the renal fraction of studies parameters provide sustained benefit in the follow-up time, above 30%.
The wet day and residual renal function are determinants in the achievement of the objective dose of dialysis, as well as in the water and sodium balance. The cause of chronic kidney disease (CKD) does not seem to influence the cleansing effectiveness of the technique.
Las infecciones continúan siendo un problema relevante en el paciente trasplantado renal, en especial las infecciones virales.
La infección por el parvovirus humano B19 causa anemia refractaria ...grave, pancitopenia y microangiopatía trombótica. Dicha infección se diagnostica mediante el análisis de la reacción en cadena de la polimerasa (PCR) en sangre y por la presencia de proeritroblastos gigantes típicos en la médula ósea.
Presentamos el caso clínico de un varón de 65 años con trasplante renal de donante cadáver en septiembre de 2014. A los 38 días del trasplante comienza con anemia progresiva y resistente a los agentes estimulantes de la eritropoyesis. A los 64 días se produce hipertermia, con deterioro progresivo de su estado general. La serología vírica resultó negativa, al igual que la PCR inicial en sangre del parvovirus humano B19. A los 4 meses y 19 días se realiza una biopsia de médula ósea en la que se observan eritroblastos gigantes con inclusiones víricas nucleares compatibles con parvovirus, por lo que se realiza una PCR en dicho tejido que confirma el diagnóstico. Una segunda PCR en sangre resultó positiva. Tras el tratamiento con inmunoglobulinas intravenosas (IGIV) y la suspensión temporal del micofenolato de mofetilo, se produce una remisión completa de la enfermedad, aunque persistía positiva la PCR para el parvovirus B19 en sangre, lo que hace necesario vigilar probables recidivas.
Infections remain an issue of particular relevance in renal transplant patients, particularly viral infections.
Human parvovirus B19 infection causes severe refractory anaemia, pancytopenia and thrombotic microangiopathy. Its presence is recognized by analysing blood polymerase chain reaction (PCR) and by the discovery of typical giant proerythroblasts in the bone marrow.
We report the case of a 65 year-old man with a history of deceased donor renal transplant in September 2014. At 38 days after the transplant, the patient presented progressive anaemia that was resistant to erythropoiesis-stimulating agents. At 64 days after transplant, hyperthermia occurred with progressive deterioration of the patient's general condition. The viral serology and the first blood PCR for human parvovirus B19 were both negative. At 4 months and 19 days after, a bone marrow biopsy was conducted, showing giant erythroblasts with nuclear viral inclusions that were compatible with parvovirus; a PCR in the tissue confirmed the diagnosis. A second blood PCR was positive for parvovirus. After treatment with intravenous immunoglobulin and the temporary discontinuation of mycophenolate mofetil, a complete remission of the disease occurred, although the blood PCR for parvovirus B19 remained positive, so monitoring is necessary for future likely recurrence.
Although clinical presentation of fibrillary glomerulonephritis is similar to most forms of glomerulonephritis, it is usually difficult to make the diagnosis. Clinical manifestations include ...proteinuria, microscopic haematuria, nephrotic syndrome, and impairment of renal function. A diagnosis of fibrillary glomerulonephritis is only confirmed by renal biopsy and it must comprise electronmicroscopy-verified ultrastructural findings. We report four cases between 45–50 years old with documented type 2 diabetes mellitus (T2DM) and arterial hypertension. All patients were found to have fibrils on kidney biopsy. The differential diagnosis of fibrils in the setting of diabetes mellitus is also discussed.
•Involving users on eHealth approaches from inception is crucial to guarantee success on real settings.•All kind of end-users should be identified carefully to develop health solutions targeted to ...the biggest audience possible.•User-centred design and development of eHealth solutions provides several benefits but disadvantages too.
Despite the promising benefits of the e-Health approaches (including provide technology-based healthcare services to anyone, anytime, and anywhere), few solutions are adopted in daily practice. User acceptance is one of the major obstacles that hinder the success of technology approaches. End-users often stress misalignments among their problems and the solutions that technology systems aim to solve. In other cases, systems developed are unfriendly or unadjusted to the daily practice of clinicians or patient’s life. To maximize user acceptance, the relevance of adopting user-centred design and development techniques is well-known. However, users are often assumed to be a homogeneous group with the same set of requirements, what leads to an ineffective identification and addressment of user requirements. Furthermore, usability and accessibility issues must be carefully addressed to guarantee also the right alignment of solutions with user needs.
to develop an e-Health system for renal patients at home by adopting user-centred design practices, usability and accessibility standards.
users were categorized in four different groups (i.e., digital patients/caregivers, non-digital patients/caregivers, clinicians and nurses) and a sample was included in the design and development team. Questionnaires and interviews were used to identify user requirements and assess prototypes.
Requirements were considered for every kind of user, what resulted on a multi-faceted e-Health system implying different technologies and functionalities regarding to each target user.
Identification and continuous involvement of all kind of users allow their needs to be properly understood and addressed by technology, raising user acceptance of the final product.
A 46-year-old female patient presenting with acute interstitial nephritis and anterior uveitis was admitted. The renal biopsy disclosed the presence of interstitial nephritis, confirming the clinical ...diagnosis of tubulointerstitial nephritis and uveitis (TINU) syndrome. Treatment with oral steroids was started, with prompt improvement of symptoms and laboratory abnormalities.
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