14.2 Causes of early death in juvenile onset systemic lupus erythematosus (JSLE)

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Septic shock, hyperferritinemic syndrome, and multiple organ dysfunction without respiratory failure in a patient with disseminated histoplasmosis and advanced HIV disease

AIDS-related disseminated histoplasmosis (DH) can cause septic shock and multiorgan dysfunction with mortality rates of up to 80%. A 41-year-old male presented with fever, fatigue, weight loss, disseminated skin lesions, low urine output, and mental confusion. Three weeks before admission, the patient was diagnosed with HIV infection, but antiretroviral therapy (ART) was not initiated. On day 1 of admission, sepsis with multiorgan dysfunction (acute renal failure, metabolic acidosis, hepatic failure, and coagulopathy) was identified. A chest computed tomography showed unspecific findings. Yeasts suggestive of Histoplasma spp. were observed in a routine peripheral blood smear. On day 2, the patient was transferred to the ICU, where his clinical condition progressed with reduced level of consciousness, hyperferritinemia, and refractory septic shock, requiring high doses of vasopressors, corticosteroids, mechanical ventilation, and hemodialysis. Amphotericin B deoxycholate was initiated. On day 3, yeasts suggestive of Histoplasma spp. were observed in the bone marrow. On day 10, ART was initiated. On day 28, samples of peripheral blood and bone marrow cultures revealed Histoplasma spp. The patient stayed in the ICU for 32 days, completing three weeks of intravenous antifungal therapy. After progressive clinical and laboratory improvement, the patient was discharged from the hospital on oral itraconazole, trimethoprim-sulfamethoxazole, and ART. This case highlights the inclusion of DH in the differential diagnosis of patients with advanced HIV disease, septic shock and multiorgan dysfunction but without respiratory failure. In addition, it provides early in-hospital diagnosis and treatment and comprehensive management in the ICU as determining factors for a good outcome

Aspiration of parenteral nutrition – a previously unreported complication of central venous access in an infant: a case report

Introduction: The insertion of percutaneous central venous catheters is a common procedure in neonatal intensive care nurseries. Placement of the catheter tip in a large central vein is most desirable. Occasionally, due to difficult venous access, catheter tips are left in places that are less than ideal. Case presentation: A female infant with a complicated gastroschisis developed signs of short bowel syndrome post surgery. She was treated with a combination of parenteral nutrition and enteral feeds. A central venous line was inserted through a scalp vein. The tip was noted to be in a vessel at the level of the mandible. She subsequently became unwell with large milky pharyngeal aspirates and episodes of bradycardia. Chest radiography revealed aspiration. The central venous line was removed because of presumed extravasation. This is the first reported case of parenteral nutrition extravasation into the pharynx causing aspiration in an infant. Conclusion: This complication may have been prevented by recognising that the tip of the catheter was not correctly placed. When catheters are in unusual positions it may be useful to obtain a second radiograph from a different angle or an ultrasound scan to confirm the positioning of the catheter tip

Aspiration of parenteral nutrition – a previously unreported complication of central venous access in an infant: a case report

Introduction: The insertion of percutaneous central venous catheters is a common procedure in neonatal intensive care nurseries. Placement of the catheter tip in a large central vein is most desirable. Occasionally, due to difficult venous access, catheter tips are left in places that are less than ideal. Case presentation: A female infant with a complicated gastroschisis developed signs of short bowel syndrome post surgery. She was treated with a combination of parenteral nutrition and enteral feeds. A central venous line was inserted through a scalp vein. The tip was noted to be in a vessel at the level of the mandible. She subsequently became unwell with large milky pharyngeal aspirates and episodes of bradycardia. Chest radiography revealed aspiration. The central venous line was removed because of presumed extravasation. This is the first reported case of parenteral nutrition extravasation into the pharynx causing aspiration in an infant. Conclusion: This complication may have been prevented by recognising that the tip of the catheter was not correctly placed. When catheters are in unusual positions it may be useful to obtain a second radiograph from a different angle or an ultrasound scan to confirm the positioning of the catheter tip

Ordered mesoporous silica materials for protein adsorption

Lysozyme and BSA were used, as model proteins of considerably different dimensions, in order to evaluate the influence of the distinct pore structural characteristics of three types of ordered mesoporous silica materials (MCF, SBA-15 and MCM-41) on protein adsorption. Characterisation by X ray diffraction and nitrogen adsorption at 77K revealed the typical pore structural features of each type of material. The maximum of the pore size distributions indicated that the width of the windows of MCF (2) (mesitylene/P123 of 2) was larger than the pore diameter of the unidirectional tubular pores of SBA-15. All the materials presented similar small external surface areas but high pore volumes, with that of MCF (2) being the highest. The adsorption of lysozyme at pH=8 increased in the order MCM-41<< SBA-15< MCF (2), and the uptakes were well above those of BSA at pH=5. Although BSA is not completely excluded from the mesopores of SBA-15 and MCF (2), as happens with MCM-41, the adsorption occurs to a very limited extent. The overall behaviour of these SBA-15 and MCF (2) samples was not significantly different and both revealed potential for the separation of these proteins

Isogeometric analysis for fluid shear stress in cancer cells

Este trabalho foi financiado pelo Concurso Anual para Projetos de Investigação, Desenvolvimento, Inovação e Criação Artística (IDI&CA) 2018 do Instituto Politécnico de Lisboa. Código de referência IPL/2018/IGACFC_ISELThe microenvironment of the tumor is a key factor regulating tumor cell invasion and metastasis. The effects of physical factors in tumorigenesis is unclear. Shear stress, induced by liquid flow, plays a key role in proliferation, apoptosis, invasion, and metastasis of tumor cells. The mathematical models have the potential to elucidate the metastatic behavior of the cells’ membrane exposed to these microenvironment forces. Due to the shape configuration of the cancer cells, Non-uniform Rational B-splines (NURBS) lines are very adequate to define its geometric model. The Isogeometric Analysis allows a simplified transition of exact CAD models into the analysis avoiding the geometrical discontinuities of the traditional Galerkin traditional techniques. In this work, we use an isogeometric analysis to model the fluid-generated forces that tumor cells are exposed to in the vascular and tumor microenvironments, in the metastatic process. Using information provided by experimental tests in vitro, we present a suite of numerical experiments which indicate, for standard configurations, the metastatic behavior of cells exposed to such forces. The focus of this paper is strictly on geometrical sensitivities to the shear stress’ exhibition for the cell membrane, this being its innovation.info:eu-repo/semantics/publishedVersio

Differences sustained between diffuse and limited forms of juvenile systemic sclerosis in expanded international cohort. www.juvenile-scleroderma.com

OBJECTIVES: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (jSSc) patients in the international Juvenile SSc Inception Cohort (jSScC), compare these characteristics between the classically defined diffuse (dcjSSc) and limited cutaneous (lcjSSc) subtypes, and among those with overlap features. METHODS: A cross-sectional study was performed using baseline visit data. Demographic, organ system evaluation, treatment, and patient and physician reported outcomes were extracted and summary statistics applied. Comparisons between dcjSSc and lcSSc subtypes and patients with and without overlap features were performed using Chi-square and Mann Whitney U-tests. RESULTS: At data extraction 150 jSSc patients were enrolled across 42 centers, 83% were Caucasian, 80% female, dcjSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between dcjSSc and lcjSSc regarding the modified Rodnan Skin Score, presence of Gottron's papules, digital tip ulceration, 6 Minute walk test, composite pulmonary and cardiac involvement. All more frequent in dcSSc except for cardiac involvement. DcjSSc patients had significantly worse scores for physician rated disease activity and damage. A significantly higher occurrence of Gottron's papules, musculoskeletal involvement and composite pulmonary involvement, and significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. CONCLUSION: Results from a large international jSSc cohort demonstrate significant differences between dcjSSc and lcjSSc patients including more globally severe disease and increased frequency of ILD in dcjSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease