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Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a recently recognized disorder characterized by vascular lesions marked by distinct endothelial proliferation. Lesions affect multiple tissues, and MLT can be associated with refractory thrombocytopenia resulting in life-threatening bleeding. Diagnosing MLT may be challenging given its rarity and phenotypic variability. There is no consensus on the optimal management or treatment duration. We report a 4-month-old male who presented with multiple vascular malformations involving the gastrointestinal tract, lung, bones, choroid plexus, and spleen, with minimal cutaneous involvement and no thrombocytopenia. Wedge resection of a pulmonary nodule was strongly positive for lymphatic vessel endothelial hyaluronan receptor 1 favoring MLT despite the lack of thrombocytopenia. The patient's clinical symptoms and vascular lesions improved on sirolimus therapy. We review the literature to highlight the clinical variability of MLT and discuss the diagnostic and therapeutic options for MLT.
The maxillary swing approach was introduced three decades ago in the head and neck field providing optimal surgical exposure for tumors in the nasopharyngeal and/or the retromaxillary space.
To report the clinical experience, patient surgical morbidity and survival outcomes following the introduction of the maxillary swing approach in Denmark.
A retrospective study including patients who underwent the maxillary swing approach from January 2012 - January 2020. Baseline and perioperative data, pathology, postoperative morbidity and survival outcomes were registered.
Sixteen patients were included of which 15 had a malignant tumor with different histology, while one patient had a benign tumor. Most commonly reported short-term morbidity were trismus, cheek hypoesthesia, nasopalatal fistula, lacrimation and nasal stenosis (<3 months postoperatively) improving markedly at 12 months follow-up. For patients with malignant tumors, the 5-year overall survival and recurrence-free survival rates were 60% and 66.7%, respectively.
The maxillary swing approach was safely implemented by a multidisciplinary team at a high-volume centralized head and neck cancer center in Denmark. The procedure may be considered for salvage surgery of recurrent nasopharyngeal carcinomas and selected malignant and benign tumors located in the nasopharynx and/or retromaxillary space inaccessible by other surgical modalities.
The maxillary swing approach was safely implemented by a multidisciplinary team at a high-volume centralized head and neck cancer center in Denmark. The procedure may be considered for salvage surgery of recurrent nasopharyngeal carcinomas and selected malignant and benign tumors located in the nasopharynx and/or retromaxillary space inaccessible by other surgical modalities.Background The UN's Sustainable Development Goals (SDGs) which pledge to leave no one behind for Universal health coverage (UHC) raise the importance of ensuring equitable health outcomes and healthcare delivery. As Neglected Tropical Diseases (NTDs) affect the most disadvantaged and hard to reach populations, they are considered a litmus test for Universal health coverage.Objective Here, we assess the challenges of implementing Mass Drug Administrations (MDAs) for schistosomiasis prevention and control, in a context of expanded treatment where both community and school-based distribution were carried out, assessing which groups are missed and developing strategies to enhance equity.Methods This is a qualitative study applying ethnographic observations, in-depth interviews (109) and focus group discussions (6) with key informants and other community members. Participants included community drug distributors, teachers, health workers, and implementing partners across four schistosomiasis endemic regions in Camrust at the community level and work with established partnerships and local governance structures that can support sustainable solutions for more equitable MDA campaigns.
Uremia induces various pathologic changes in the endothelium. However, there is limited information about the differences of these effects in endothelial cells originating from different parts of the vascular tree.
The effect of uremic serum obtained from patients with end stage renal failure on the gene expression and secretory activity of venous endothelial cells (VEC) and aortic endothelial cells (AEC) was studied in
culture.
In VEC, the expression of genes regulating the synthesis of von Willebrand factor (vWF) was increased by 254% (
<.005), vascular endothelial growth factor (VEGF) synthesis by 150% (
<.001), tissue plasminogen activator (t-PA) synthesis by 62% (
<.005), platelet endothelial cell adhesion molecule by 89% (
<.005), and the expression of gene regulating interleukin-6 (IL-6) synthesis was reduced. In AEC, the expression of the gene regulating synthesis of IL-6 was increased by 174% (
<.001), and the expression of the other genes was reduced. The secretion of IL-6 was reduced in VEC by 38% (
<.01) and increased in AEC by 55% (
<.005). In VEC, increased synthesis of VEGF 64% (
<.001) vWF (+34%,
<.01), and t-PA (+53%,
<.002) was observed, and in AEC it was reduced.
VEC and AEC respond in different ways after exposure to uremic serum. VEC acquires the prothrombotic phenotype, whereas in AEC the inflammatory phenotype appears.
VEC and AEC respond in different ways after exposure to uremic serum. VEC acquires the prothrombotic phenotype, whereas in AEC the inflammatory phenotype appears.Purpose To determine the feasibility of non-invasive tear break-up time (NIBUT) assessment using the Handheld tear lipid layer thickness assessment instrument and compare it with the standard tear break-up time (TBUT) test and other dry eye tests.Subjects and methods 108 subjects were enrolled, 56 with and 52 without dry eye symptoms. Schein questionnaire was used to determine the severity of dry eye symptoms. Ocular signs were assessed by NIBUT, TBUT, lipid layer thickness (LLT), lid-parallel conjunctival folds (LIPCOF), conjunctival hyperemia, and corneal staining.Results Median NIBUT and TBUT, and other clinical test values significantly differed among the dry eye symptoms group and control group. NIBUT yielded the most significant difference between the groups (NIBUT 7 sec vs. 17.5 sec, p less then .001, Z = 5.94; TBUT 5 sec vs. 10 sec, p less then .001, Z = 4.38; LLT p = .007; LIPCOF p less then .001, conjunctival hyperemia p less then .047, corneal staining p less then .010). buy Fenretinide Spearman's test showed a significant correlation between NIBUT and TBUT (p less then .
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