ABSTRACT Introduction: Chronic recurrent multifocal osteomyelitis (CRMO), also called chronic non-bacterial osteomyelitis, is an autoinflammatory disease characterized by bone involvement, recurrent ...flare-ups, and the lack of microbiological isolation. It is a diagnosis of exclu sion, and the fundamental basis of treatment is non-steroidal anti-inflammatory drugs. The objective of the study is to describe our experience as a result of three girls diagnosed with CRMO, highlighting the clinical presentation, the findings in the complementary tests, the treatment, and the evolution of the disease. Patients and methods: Retrospective chart review of children with CRMO in the last 5 years, being followed-up in a pediatric rheumatology clinic in a tertiary center. Results: The cases are presented of 3 patients diagnosed with CRMO, all of them young girls, with a mean age of 11 years, who consulted due to pain and functional impotence. It was in single location in two cases, and the other with several sources of pain, at cervi cal and lumbar level, associated with weakness of the upper and lower limbs. Two of the cases received antibiotic treatment. One girl responded to treatment with anti-inflammatory drugs and another required combining corticosteroids. The remaining case, in addition to anti-inflammatory drugs and corticosteroids, required intravenous pamidronate. Conclusions: With this study, and despite the small sample size, the aim was to highlight the importance of this, in many cases unknown and underdiagnosed, pathology, and to stress the importance of establishing a diagnostic and therapeutic protocol for the correct approach to this disease.
La osteomielitis crónica multifocal recurrente (OCMR), también conocida como osteomielitis crónica no bacteriana, es una enfermedad autoinflamatoria caracterizada por afectación ósea, de curso en ...brotes y en ausencia de aislamiento microbiológico. El diagnóstico es de exclusión y el pilar fundamental del tratamiento son los antiinflamatorios no esteroideos (AINES). El objetivo del estudio es describir nuestra experiencia de tres niñas diagnosticadas de OCMR, destacando la presentación clínica, los hallazgos en las pruebas complementarias, el tratamiento y la evolución de la enfermedad.
Revisión retrospectiva de historias clínicas de niños diagnosticados de OCMR en los últimos cinco años, en seguimiento en consulta de reumatología pediátrica de un hospital terciario.
Presentamos tres pacientes diagnosticadas de OCMR, todas ellas mujeres adolescentes, con media de edad de 11 años. Consultaron por dolor e impotencia funcional, dos en una única localización y la otra por varios focos de dolor, a nivel cervical y lumbar, asociando debilidad de miembros superiores e inferiores. Con respecto al tratamiento, dos recibieron tratamiento antibiótico. Una niña respondió a antiinflamatorios; otra precisó asociar corticoides, y la restante, además de antiinflamatorios y corticoides, necesitó pamidronato intravenoso.
Con este estudio y a pesar del pequeño tamaño muestral, se pretende resaltar la importancia de esta patología, en muchos casos desconocida e infradiagnosticada, e insistir en la importancia de establecer un protocolo diagnóstico y terapéutico para su correcto abordaje.
Chronic recurrent multifocal osteomyelitis (CRMO), also called chronic non-bacterial osteomyelitis, is an autoinflammatory disease characterised by bone involvement, recurrent flare-ups, and the lack of microbiological isolation. It is a diagnosis of exclusion, and the fundamental basis of treatment is non-steroidal anti-inflammatory drugs. The objective of the study is to describe our experience as a result of three girls diagnosed with CRMO, highlighting the clinical presentation, the findings in the complementary tests, the treatment, and the evolution of the disease.
Retrospective chart review of children with CRMO in the last 5 years, being followed-up in a paediatric rheumatology clinic in a tertiary centre.
The cases are presented of 3 patients diagnosed with CRMO, all of them young girls, with a mean age of 11 years, who consulted due to pain and functional impotence. It was in single location in two cases, and the other with several sources of pain, at cervical and lumbar level, associated with weakness of the upper and lower limbs. Two of the cases received antibiotic treatment. One girl responded to treatment with anti-inflammatory drugs and another required combining corticosteroids. The remaining case, in addition to anti-inflammatory drugs and corticosteroids, required intravenous pamidronate.
With this study, and despite the small sample size, the aim was to highlight the importance of this, in many cases unknown and underdiagnosed, pathology, and to stress the importance of establishing a diagnostic and therapeutic protocol for the correct approach to this disease.
Las enfermedades por alteraciones de la biosíntesis de coenzima Q10, como la glomerulopatía secundaria a mutación en el gen ADCK4, son las únicas enfermedades mitocondriales con tratamiento médico ...efectivo. Presentamos el caso de un varón de 8 años con proteinuria en rango nefrótico, sin síntomas sistémicos y con nefrocalcinosis bilateral ecográfica. Presenta una mutación en homocigosis en el gen ADCK4, c.439T>C (p.Cys147Arg), no descrita previamente como patogénica. Tras el tratamiento con coenzima Q10 presenta un descenso de la proteinuria mayor del 50%. Esta glomerulopatía puede manifestarse como síndrome nefrótico corticorresistente de inicio tardío. El tratamiento con coenzima Q10 es útil en los estadios iniciales.
Long-term adaptive immune memory has been reported among immunocompetent individuals up to eight months following SARS-CoV-2 infection. However, limited data is available in convalescent patients ...with a solid organ transplant. To investigate this, we performed a thorough evaluation of adaptive immune memory at different compartments (serological, memory B cells and cytokine IFN-γ, IL-2, IFN-γ/IL12 and IL-21 producing T cells) specific to SARS-CoV-2 by ELISA and FluoroSpot-based assays in 102 convalescent patients (53 with a solid organ transplants (38 kidney, 5 liver, 5 lung and 5 heart transplant) and 49 immunocompetent controls) with different clinical COVID-19 severity (severe, mild and asymptomatic) beyond six months after infection. While similar detectable memory responses at different immune compartments were detected between those with a solid organ transplant and immunocompetent individuals, these responses were predominantly driven by distinct COVID-19 clinical severities (97.6%, 80.5% and 42.1%, all significantly different, were seropositive; 84% vs 75% vs 35.7%, all significantly different, showed IgG-producing memory B cells and 82.5%, 86.9% and 31.6%, displayed IFN-γ producing T cells; in severe, mild and asymptomatic convalescent patients, respectively). Notably, patients with a solid organ transplant with longer time after transplantation did more likely show detectable long-lasting immune memory, regardless of COVID-19 severity. Thus, our study shows that patients with a solid organ transplant are capable of maintaining long-lasting peripheral immune memory after COVID-19 infection; mainly determined by the degree of infection severity.
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The demand for multifunctional requirements in aerospace, military, automobile, sports, and energy applications has encouraged the investigation of new composite materials. This study focuses on the ...development of multiwall carbon nanotube (MWCNT) filled polypropylene composites and carbon nanofiber composite mats. The developed systems were then used to prepare interlayered composites that exhibited improved electrical conductivity and electromagnetic interference (EMI) shielding efficiency. MWCNT-carbon nanofiber composite mats were developed by centrifugally spinning mixtures of MWCNT suspended in aqueous poly(vinyl alcohol) solutions. The developed nanofibers were then dehydrated under sulfuric acid vapors and then heat treated. Interlayered samples were fabricated using a nanoreinforced polypropylene composite as a matrix and then filled with carbon fiber composite mats. The in-plane and through-plane electrical conductivity of an eight-layered flexible carbon composite (0.65 mm thick) were shown to be 6.1 and 3.0 × 10
S·cm
, respectively. The EMI shielding effectiveness at 900 MHz increased from 17 dB for the one-layered composite to 52 dB for the eight-layered composite. It was found that the reflection of the electromagnetic waves was the dominating mechanism for EMI shielding in the developed materials. This study opens up new opportunities for the fabrication of novel lightweight materials that are to be used in communication systems.
Objective:To evaluate the physical compatibility between intravenous magnesium sulfate and potassium and sodium phosphate, a common electrolyte intravenous supplementation in pediatric intensive care ...units. Study design: Magnesium sulfate was mixed separately with potassium phosphate and sodium phosphate at ratios of 1:1, 1:4, and 4:1. Binary mixtures were prepared, in triplicate and under sterile conditions, by permuting the order of addition. The undiluted pure drugs were used as controls for possible sequence effects. Visual changes, turbidity, and pH were assessed immediately after mixing (baseline) and at 4 and 24 hours. Two observers performed visual changes by naked-eye visual inspection in order to search visible haze, particulate matter, gas formation, or color change. Turbidity was measured by nephelometry and incompatibility was defined as an increase of ≥0.5 nephelometric turbidity units (NTU) from baseline. pH was measured using a portable pH meter and incompatibility was defined as a variation of >1 pH unit during the observation period. Results: None of the admixtures exhibited visual changes or significant variations in turbidity (increases of ≥0.5 in nephelometric turbidity units) or pH (changes of >1 unit) during the observation period and neither compared with baseline. Conclusion: In this study, no visual changes were observed, and turbidity and pH evaluated by instrumental methods remained within acceptable limits and showed no significant variations from baseline, therefore no physical incompatibility between magnesium sulfate and potassium or sodium phosphate was found.
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