يعرض 1 - 10 نتائج من 27 نتيجة بحث عن '"Mika M. Tabata"', وقت الاستعلام: 1.39s تنقيح النتائج
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    دورية أكاديمية

    المصدر: Clinical Case Reports, Vol 7, Iss 12, Pp 2491-2494 (2019)

    الوصف: Abstract Chronic cutaneous graft‐vs‐host disease (GVHD) has several atypical variants. We describe two cases of GVHD with clinical and histopathologic features of pityriasis rubra pilaris (PRP), which responded to additional immunosuppression. Recognition of this newly described PRP‐like clinical presentation of GVHD may prompt early consideration of additional steroid‐sparing therapies.

    وصف الملف: electronic resource

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    دورية أكاديمية
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    دورية أكاديمية

    المصدر: JCI Insight, Vol 5, Iss 16 (2020)

    مصطلحات موضوعية: Dermatology, Neuroscience, Medicine

    الوصف: Neurofibromatosis type 1 (NF1) is a rare genetic disorder, characterized by the development of benign and malignant nerve tumors. Although all individuals with NF1 harbor genetic alterations in the same gene, the clinical manifestations of NF1 are extremely heterogeneous even among individuals who carry identical genetic defects. In order to deepen the understanding of phenotypic manifestations in NF1, we comprehensively characterized the prevalence of 18 phenotypic traits in 2051 adults with NF1 from the Children’s Tumor Foundation’s NF1 registry. We further investigated the coassociation of traits and found positive correlations between spinal neurofibromas and pain, spinal neurofibromas and scoliosis, spinal neurofibromas and optic gliomas, and optic gliomas and sphenoid wing dysplasia. Furthermore, with increasing numbers of cutaneous neurofibromas, the odds ratio of malignant peripheral nerve sheath tumor increased. Phenotypic clustering revealed 6 phenotypic patient cluster subtypes: mild, freckling predominant, neurofibroma predominant, skeletal predominant, late-onset neural severe, and early-onset neural severe, highlighting potential phenotypic subtypes within NF1. Together, our results support potential shared molecular pathogenesis for certain clinical manifestations and illustrate the utility of disease registries for understanding rare diseases.

    وصف الملف: electronic resource

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    دورية أكاديمية

    المصدر: Nutrients, Vol 7, Iss 6, Pp 5051-5064 (2015)

    الوصف: Continuous infusion systems used for enteral nutrition support in the neonatal intensive care unit deliver as little as 60% of the fat in human milk to the neonate. This study determined the effect of mixing common feedings for preterm infants in the feeding bag and tubing on fat losses during enteral feeding. Laboratory models were developed to assess the contribution of various mixing techniques to delivered fat content. Fat content was measured periodically during feeding and compared to baseline measurements. A multistage approach incorporating a feeding bag inverter and a tubing circulation loop delivered >90% of milk fat when used in conjunction with a commercial continuous infusion system. With unfortified human milk, this approach delivered 91.9% ± 1.5% of fat content over a one hour feed, significantly greater (p < 0.01) than 77.5% ± 2.2% delivered by continuous infusion controls (Mean ± SEM). With fortified human milk, this approach delivered 92.1% ± 2.4% of fat content, significantly greater (p < 0.01) than 79.4% ± 1.0% delivered by a non-adapted infusion system (Mean ± SEM). Mixing human milk during continuous infusion improves fat delivery, which may improve nutrition and growth outcomes in low birth weight neonates.

    وصف الملف: electronic resource

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    المصدر: BMJ case reports. 16(1)

    الوصف: A woman in her 70s was referred for a painless plaque on the shin, present for 2 years and progressing in thickness. Examination revealed a large erythematous to violaceous indurated plaque with cobblestone appearance. Biopsy revealed an inflammatory infiltrate of neutrophils with scattered histiocytes, lymphocytes, eosinophils and plasma cells interspersed with areas of lamellar fibrosis and focal areas of vascular damage, suggestive of a localised chronic fibrosing vasculitis of the skin. Localised chronic fibrosing vasculitis is a rare dermatosis, typically presenting as ulcerated violet-red nodules, which can appear histologically similar to erythema elevatum diutinum (EED), which typically presents as red-brown annular plaques. EED may have a predominance of neutrophils and granulomas, while chronic fibrosing vasculitis may have a sparse infiltrate of mixed inflammatory cells without granulomas. While dapsone is a first-line treatment for EED, there are no formal guidelines on the treatment of localised chronic fibrosing vasculitis. Given the neutrophils in this sample and similarities with EED, this patient was treated with oral dapsone, resulting in plaque improvement.

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    المصدر: Journal of Cutaneous Pathology. 47:1042-1045

    الوصف: We report a case of differentiation syndrome in a patient receiving the IDH1 inhibitor ivosidenib, with skin biopsy showing isocitrate dehydrogenase (IDH) R132H-mutated leukemia cutis. A 72-year-old man with IDH1-mutated acute myeloid leukemia (AML), status-post allogeneic cell transplantation, on ivosidenib for six months, was admitted for culture-negative neutropenic fever, pink and purpuric plaques and patches on the legs, abdomen and back, edema, hypotension, and shortness of breath. Skin biopsy revealed an infiltrate of atypical, immature, myeloperoxidase-positive mononuclear cells compatible with leukemia cutis or Sweet syndrome. Although dermal edema and interstitial neutrophilic infiltrate with karyorrhexis characteristic of Sweet syndrome were not seen, the atypical cells lacked expression of CD117 and CD34, which were expressed in the original leukemia. Additional immunohistochemical staining of suspected blasts was strongly positive for IDH1 R132H, suggesting a diagnosis of leukemia cutis. As the immunophenotype of blasts in skin infiltrates can significantly differ from the immunophenotype seen in blood and bone marrow, this case demonstrates that mutation-specific antibodies such as anti-IDH1 R132H may be useful to help distinguish malignant from non-malignant infiltrates in the skin. Furthermore, differentiation syndrome may demonstrate histologic features of leukemia cutis on skin biopsy. This article is protected by copyright. All rights reserved.

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    المصدر: SKIN The Journal of Cutaneous Medicine. 3:405-409

    الوصف: Importance: Managing chronic skin disease is often frustrating for both providers and patients, sometimes resulting in delayed diagnosis, inadequate therapy, and inconsistent care.Objective: This study performs stakeholder analyses to identify unmet clinical needs in chronic skin disease management. Methods: Survey of 33 providers and 25 patients at a Stanford Health Care Dermatology department.Results: When evaluating a chronic skin condition such as psoriasis, 79% of dermatologists rely solely on subjective documentation (gestalt, body surface area, descriptive exam). Objective documentation (photographs or scoring assessment tools) is used by 21% of providers upon initial assessment and by 7% of providers to assess change in disease between office visits. While 83% of providers were comfortable assessing change in disease severity based on prior document by oneself, only 31% were comfortable assessing change based on prior documentation by another provider (p

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    المصدر: SKIN The Journal of Cutaneous Medicine. 3:309-320

    الوصف: Introduction: Currently there are no portable solutions to robustly document and longitudinally monitor dynamically changing chronic skin conditions. This study set out to engineer and test a mobile-based 3D imaging solution for chronic skin diseases to enhance clinical workflow and patient care.Methods: SkinSpecs uses smartphone-captured videos of patients’ skin disease and renders 3D true-to-life models that were evaluated by Stanford Health Care dermatologists.Results: We utilized video input to accurately reconstruct interactive 3D models of 16 different skin conditions from 31 patients. Assessment of SkinSpecs 3D reconstruction is faster (p