Effectiveness of the treatment with intravenous pamidronate in calcinosis in juvenile dermatomyositis.

OBJECTIVE: Calcinosis is a frequent finding in up to 40% of children with juvenile dermatomyositis (JDM). Different treatments (aluminum hydroxide, diltiazem, probenecid, alendronate, etc.) have been used in an attempt to clear calcinosis and to avoid the onset of new calcium deposition, but none has been clearly effective. Pamidronate is a nitrogen-containing bisphosphonate with a potent inhibiting bone resorption effect that has been used to treat osteoporosis in children. We report three children with JDM who developed calcinosis and who received intravenous pamidronate with good results. METHODS: All three patients met the Bohan and Peter diagnostic criteria for JDM. Intravenous pamidronate was given at 1 mg/kg/day on three consecutive days every three months according to the protocol established by Glorieux et al. for osteoporosis treatment in osteogenesis imperfecta. RESULTS: The calcinosis which developed in all three patients improved. No important adverse events were observed. CONCLUSION: In all three cases, calcinosis significantly decreased, and even totally cleared in patient 1. Total clearance of pre-existing calcinosis in JDM with pamidronate therapy has not been previously described with any of the aforementioned treatments. The advantage of treatment with pamidronate compared to treatment with alendronate is that intravenous administration does not produce esophagitis, the most frequent adverse event when orally administering bisphosphonates. Our results strongly suggest that therapy with intravenous pamidronate in conjunction with good disease control with DMARD therapy is an apparently safe and effective treatment for calcinosis management in JDM.

Revisiting the case of R Monocerotis: Is CO removed at R < 20 au?⋆.

CONTEXT: To our knowledge, R Mon is the only B0 star in which a gaseous Keplerian disk has been detected. However, there is some controversy about the spectral type of R Mon. Some authors propose that it could be a later B8e star, where disks are more common. AIMS: Our goal is to re-evaluate the R Mon spectral type and characterize its protoplanetary disk. METHODS: The spectral type of R Mon has been re-evaluated using the available continuum data and UVES emission lines. We used a power-law disk model to fit previous 12CO 1→0 and 2→1 interferometric observations and the PACS CO data to investigate the disk structure. Interferometric detections of 13CO J=1→0, HCO+ 1→0, and CN 1→0 lines using the IRAM Plateau de Bure Interferometer (PdBI) are presented. The HCN 1→0 line was not detected. RESULTS: Our analysis confirms that R Mon is a B0 star. The disk model compatible with the 12CO 1→0 and 2→1 interferometric observations falls short of predicting the observed fluxes of the 1431 CO lines suggest the existence of a region empty of CO at R≲20 au in the proto-planetary disk. The intense emission of the HCO+ and CN lines shows the strong influence of UV photons on gas chemistry. CONCLUSIONS: The observations gathered in this paper are consistent with the presence of a transition disk with a cavity of R in ≳20 au around R Mon. This size is similar to the photoevaporation radius that supports the interpretation that UV photoevaporation is main disk dispersal mechanism in massive stars.

Método sensible para monitorizar la migración de las células madre mesenquimales de la médula ósea en modelos murinos / A sensitive method for monitoring the migration of mesenchymal stem cells from bone marrow in murine models

OBJETIVO: Las células madre mesenquimales (MSCs) son atractivas en la terapia regenerativa de patologías humanas. En los modelos murinos, en los que se trasplantan MSCs humanas, es muy importante poder distinguir el origen de las MSCs identificadas en los órganos de ratones. El objetivo de este estudio fue determinar el rendimiento del análisis basado en PCR de secuencias Alu humanas para detectar ADN humano después de la infusión de células madre de médula ósea humana (hBMSCs) en ratones inmunodeficientes. MATERIAL Y MÉTODO: Las hBMSCs se obtuvieron de la cabeza femoral de pacientes sometidos a cirugía de reemplazo de cadera. Se infundieron 106 hBMSCs por vía intravenosa mediante inyección en el seno retro-orbitario de ratones NOD/SCID. Después se evaluó la presencia de ADN humano en pulmón, hígado y hueso. RESULTADOS: En mezclas de ADN in vitro, el ADN humano se detectó fácilmente con una buena relación logarítmica-lineal. De manera similar, cuando se mezclaron osteoblastos humanos y de ratón, se detectaron fácilmente 1-10 células humanas entre 105 células de ratón. Asimismo, se detectó el ADN humano en los pulmones 1 y 7 días después de las infusiones celulares en ratones NOD/SCID. Sin embargo, el ADN humano se detectó de manera inconsistente en el hígado y los huesos. CONCLUSIÓN: La detección de secuencias Alu es un procedimiento eficaz para detectar ADN humano. Los resultados confirman que la mayoría de las hBMSCs inyectadas por vía intravenosa quedan atrapadas en los pulmones. Por lo tanto, de cara al tratamiento de trastornos esqueléticos, se necesitan procedimientos para aumentar la migración de dichas células al hueso

OBJETIVE: Mesenchymal stem cells (MSCs) are commonly used in regenerative therapy of human diseases. In murine models, in which human MSCs are transplanted, distinguishing the origin of the identified MSCs in the organs of mice is important. The objective of this study was to determine the performance of PCR-based analysis of human Alu sequences to detect human DNA after infusion of human bone marrow stem cells (hBMSCs) in immunodeficient mice. MATERIAL AND METHOD: HBMSCs were obtained from the femoral head of patients undergoing hip replacement surgery. 106 hBMSCs were infused intravenously by injection into the retro-orbital sinus of NOD/SCID mice. The presence of human DNA in lung, liver and bone was then assessed. RESULTS: In in vitro DNA mixtures, human DNA was easily detected with a good logarithmic-linear relationship. Similarly, when human and mouse osteoblasts were mixed, 1-10 cells were easily detected among 105 mouse cells. Likewise, human DNA was detected in the lungs 1 and 7 days after cell infusions in NOD/SCID mice. However, human DNA was inconsistently detected in the liver and bones. CONCLUSION: Detecting Alu sequences is an effective procedure to observe human DNA. The results confirm that most intravenously injected hBMSCs are trapped in the lungs. Thus, for the treatment of skeletal disorders, procedures are needed to increase the migration of these cells to the bone

Cómo diagnosticamos y tratamos una enfermedad reumática: casos clínicos interactivos / How do we diagnose and treat a pediatric rheumatic disease: interactive cases report

En este seminario se trata el abordaje diagnóstico y terapéutico inicial de las enfermedades reumáticas pediátricas a través de la resolución interactiva de unos casos clínicos representativos de la artritis idiopática juvenil en sus distintas formas de expresión clínica, por ser esta la enfermedad más frecuente en esta especialidad. Se repasan los últimos avances en el diagnóstico y tratamiento de interés para el pediatra de Atención Primaria (AU)

This seminar approaches the initial diagnosis and treatment of juvenile idiopathic arthritis, the most frequent rheumatic disease in children. We present interactive cases report where the different clinical forms of juvenile idiopathic arthritis are distinguished. We review the recent advances in diagnosis and treatment of interest for the primary care pediatrician (AU)

Pielonefritis aguda con síntomas compatibles con artritis de cadera / Acute pyelonephritis with symptoms compatible with arthritis of the hip

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