- ItemOpen AccessPattern dystrophies in patients treated with deferoxamine. Report of two cases and review of the literatureBackground: Deferoxamine (DFO) is one of the most commonly used chelation treatments for transfusional hemosiderosis. Pattern dystrophies constitute a distinct entity of retinal disorders that has been occasionally identified in association with deferoxamine. Case presentation: We report two cases of bilateral macular pattern dystrophy in transfusion dependent patients undergoing chronic chelation therapy with deferoxamine due to thalassemias. Our patients were evaluated with multimodal imaging and the results are presented. Both patients had normal cone and rod responses in the full-field electroretinogram and continued the prescribed chelation therapy, after hematology consult. The patients were followed up every 3 months for 2 and 4 years respectively for possible deterioration. Their best corrected visual acuity remained stable with no anatomic change on Optical Coherence Tomography findings. Conclusion: Multimodal imaging of our patients allowed a better evaluation and possibly earlier detection of the DFO- related changes. Screening and close follow up of patients under chronic chelating therapy is important in order to promptly diagnose and manage possible toxicity either with discontinuation of the offending agent or dose modification.
- ItemOpen AccessOsteolytic lesions (brown tumors) of primary hyperparathyroidism misdiagnosed as multifocal giant cell tumor of the distal ulna andradiusBACKGROUND: Brown tumors represent a rare clinical manifestation reported in approximately 3% of patients with primary hyperparathyroidism and correspond to radiologically osteolytic lesions with well-defined borders in different parts of the skeleton. CASE PRESENTATION: We report the case of a 53-year-old white man who presented to our hospital with osteolytic lesions of his distal ulna and radius, causing pain and swelling of 2-month duration. A subsequent biopsy revealed histological features consistent with giant cell tumor and a complete resection of his distal ulna was followed, along with curettage and cementoplasty of the distal radial metaphysis. Two weeks later, he was re-admitted with diffuse musculoskeletal soreness, anorexia, constipation, nausea, and localized abdominal pain and multiple osteolytic lesions on plain radiographs. A histopathological examination of the ulna and radius specimens showed similar findings and, given the multifocality, brown tumors related to primary or secondary hyperparathyroidism was included in the differential diagnosis. A laboratory examination showed high total serum calcium (14.5 mg/dl) and low serum phosphorus and 25-hydroxyvitamin D levels. Primary hyperparathyroidism was suspected and confirmed by the elevated parathyroid hormone levels of 1453 pg/mL. At radiological work-up, using computed tomography, ultrasonography, and parathyroid subtraction technetium-99m sestamibi scintigraphy, a 4.5 × 2.5 × 3.2 cm mass emanating from the right lobe of his thyroid gland was detected, displaying extensive uptake in the right lower parathyroid gland. After appropriate medical support including hyperhydration and high doses of diuretics and diphosphonates, his laboratory profile normalized and he underwent total thyroidectomy with removal of the parathyroid glands. Our patient is now recovering 12 months after surgery, with normal values of serum parathyroid hormone and calcium levels. The lytic bone lesions have almost disappeared and no other additional orthopedic intervention was necessary. CONCLUSIONS: The present case report emphasizes the need of inclusion of brown tumors in the differential diagnosis of multifocal osteolytic bone lesions, in order to avoid harmful surgical interventions. Laboratory testing of serum phosphate, calcium levels, and parathyroid hormone levels should always be included in the routine survey of patients with multifocal osteolytic lesions.
- ItemOpen AccessGlobal avian influenza outbreaks 2010-2016 : a systematic review of their distribution, avian species and virus subtypeBACKGROUND: We conducted a systematic review to investigate avian influenza outbreaks and to explore their distribution, upon avian influenza subtype, country, avian species and other relating details as no comprehensive epidemiological analysis of global avian influenza outbreaks from 2010 to 2016 exists. METHODS: Data was collated from four databases (Scopus, Web of Science Core Correlation, PubMed and SpringerLink electronic journal) and a global electronic reporting system (ProMED mail), using PRISMA and ORION systematic approaches. One hundred seventy three avian influenza virus outbreaks were identified and included in this review, alongside 198 ProMED mail reports. RESULTS: Our research identified that the majority of the reported outbreaks occurred in 2016 (22.2%). These outbreaks were located in China (13.6%) and referred to commercial poultry farms (56.1%). The most common subtype reported in these outbreaks was H5N1 (38.2%), while almost 82.5% of the subtypes were highly pathogenic avian influenza viruses. There were differences noticed between ProMED mail and the scientific literature screened. CONCLUSIONS: Avian influenza virus has been proved to be able to contaminate all types of avian species, including commercial poultry farms, wild birds, backyard domestic animals, live poultry, game birds and mixed poultry. The study focused on wet markets, slaughterhouses, wild habitats, zoos and natural parks, in both developed and developing countries. The impact of avian influenza virus seems disproportionate and could potentially burden the already existing disparities in the public health domain. Therefore, a collaboration between all the involved health sectors is considered to be more than necessary.
- ItemOpen AccessAn optical coherence tomography study of two new generation stents with 2 biodegradable polymer carrier, eluting paclitaxel vs. biolimus-A9Background: Tissue coverage and strut apposition of drug eluting stents (DES), which can be assessed with 21 optical coherence tomography (OCT), may be associated with late stent thrombosis (LST). 22 Methods: Prospective observational angiographic and OCT follow-up at 6 months post-implantation of a 23 biolimus-A9 eluting stent (BES) vs. a paclitaxel eluting stent (PES), with biodegradable polymer carriers. The 24 primary outcome was the percent difference of uncovered struts between BESs and PESs. 25 Results: A maximum likelihood model was used for analysis, to account for data clustering. Sixteen patients 26 were treated with BES (28 lesions/4530 struts) and 16 with PES (23 lesions/4450 struts). Overall, BESs 27 compared to PESs had more uncovered [0.41% vs. 0.21%, difference estimate (DE) 0.2 (95% CI, 0.06–0.34), 28 p=0.006], malapposed [0.18% vs. 0.04%, DE 0.14 (95% CI 0.05–0.23), p=0.003], uncovered and malapposed 29 [0.08% vs. 0.026%, DE 0.057 (95% CI 0.015–0.1), p=0.01] and protruding struts [0.23% vs. 0.04%, DE 0.185 (95% 30 CI 0.1–0.27), pb0.001], and significantly lower neointimal thickness (59.3± 28.2 μm vs. 201.7± 97.5, 31 pb0.001). None of the BESs was totally covered with neointima, in contrast to 5 (21.7%) PESs (p=0.01). 32 Thrombus was detected in 1 (3.6%) BES and 5 (21.7%) PESs (p=0.05); however, no patient experienced 33 clinical stent thrombosis. 34 Conclusion: Between two stents with biodegradable polymer, OCT demonstrated that BESs had more 35 uncovered and malapposed struts compared to PESs at 6 months. This difference might be partly attributed to 36 the more potent antiproliferative properties of biolimus-A9; however, its impact on clinical outcome and on 37 the risk of LST is yet to be determined.
- ItemOpen AccessNRAS destines tumor cells to the lungsThe lungs are frequently affected by cancer metastasis. Although NRAS mutations have been associated with metastatic potential, their exact role in lung homing is incompletely understood. We cross-examined the genotype of various tumor cells with their ability for automatic pulmonary dissemination, modulated NRAS expression using RNA interference and NRAS overexpression, identified NRAS signaling partners by microarray, and validated them using Cxcr1- and Cxcr2-deficient mice. Mouse models of spontaneous lung metastasis revealed that mutant or overexpressed NRAS promotes lung colonization by regulating interleukin-8-related chemokine expression, thereby initiating interactions between tumor cells, the pulmonary vasculature, and myeloid cells. Our results support a model where NRAS-mutant, chemokine-expressing circulating tumor cells target the CXCR1-expressing lung vasculature and recruit CXCR2-expressing myeloid cells to initiate metastasis. We further describe a clinically relevant approach to prevent NRAS-driven pulmonary metastasis by inhibiting chemokine signaling. In conclusion, NRAS promotes the colonization of the lungs by various tumor types in mouse models. IL-8-related chemokines, NRAS signaling partners in this process, may constitute an important therapeutic target against pulmonary involvement by cancers of other organs.