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https://www.readbyqxmd.com/read/29675756/nodular-macroregenerative-tissue-as-a-pattern-of-regeneration-in-cholangiopathic-disorders
#1
Preston Roberts, Andrew T Trout, Jonathan R Dillman
BACKGROUND: Published case series have described central hepatic macroregenerative nodules or masses as a common feature of Alagille syndrome. Our experience suggests this regenerative pattern can be seen more generally in cholangiopathic disorders. OBJECTIVE: To define the frequency of central regenerative tissue in Alagille syndrome and other cholangiopathic disorders and to describe the typical appearance of such regenerative tissue. MATERIALS AND METHODS: We conducted a retrospective study of CT and MR imaging performed in children and young adults with cholangiopathic disorders between January 2000 and June 2016...
April 19, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29675407/pancreatic-aquaporin-7-a-novel-target-for-anti-diabetic-drugs
#2
REVIEW
Leire Méndez-Giménez, Silvia Ezquerro, Inês V da Silva, Graça Soveral, Gema Frühbeck, Amaia Rodríguez
Aquaporins comprise a family of 13 members of water channels (AQP0-12) that facilitate a rapid transport of water across cell membranes. In some cases, these pores are also permeated by small solutes, particularly glycerol, urea or nitric oxide, among other solutes. Several aquaporins have been identified in the pancreas, an exocrine and endocrine organ that plays an essential role in the onset of insulin resistance and type 2 diabetes. The exocrine pancreas, which accounts for 90% of the total pancreas, secretes daily large volumes of a near-isotonic fluid containing digestive enzymes into the duodenum...
2018: Frontiers in Chemistry
https://www.readbyqxmd.com/read/29673124/liquid-based-cytology-in-the-diagnosis-of-langerhans-cell-sarcoma-a-case-report
#3
Kazumi Iwasaki, Yasuhiro Sakai, Masaki Mori, Yoshiaki Imamura
Langerhans cell sarcoma (LCS) is an extremely rare malignant dendritic cell neoplasm with Langerhans cell differentiation. Conventional cytology, based on cell morphology alone, cannot render a cytological diagnosis of LCS because immunochemical analysis is essential to identify the Langerhans cell immunophenotype. We present a case illustrating the value of liquid-based cytology with immunocytochemistry as compared with conventional cytology, along with histological and immunohistochemical findings. A 92-year-old woman presented with a 1-month history of progressive right cervical lymphadenopathy...
April 19, 2018: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/29673064/successful-pregnancy-and-delivery-after-simultaneous-islet-kidney-transplantation
#4
Michela Assalino, Michele Podetta, Sandrine Demuylder-Mischler, Katyuska Francini, Nadine Pernin, Jean-Pierre Randin, Domenico Bosco, Axel Andres, Thierry Berney
Allogeneic islet of Langerhans transplantation is a recognized beta-cell replacement therapy for patients affected by type 1 diabetes mellitus. Type 1 diabetes mellitus is a condition associated with an increased risk of adverse outcomes for pregnant women and fetuses We report the case of a 29 year-old woman with type 1 diabetes mellitus, who underwent successful allogeneic islet transplantation with simultaneous kidney transplantation. She achieved durable insulin independence after two islet infusions. Pregnancy was desired and planned 2 years after last islet infusion...
April 19, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29672867/langerhans-and-inflammatory-dendritic-epidermal-cells-in-atopic-dermatitis-are-tolerized-towards-tlr2-activation
#5
Kazumasa Iwamoto, Tim J Stroisch, Susanne Koch, Nadine Herrmann, Nicole Leib, Thomas Bieber
BACKGROUND: The skin of atopic dermatitis (AD) patients presents a significant dysbalance of the microbiome with a high colonization by Staphylococcus aureus (S. aureus) which positively correlates with the severity of the disease. OBJECTIVE: Understanding the role of epidermal dendritic cells as link between the innate and the adaptive immune systems in AD. METHODS: Comparative phenotypic and functional analysis of TLR2 on Langerhans cells (LC) and inflammatory dendritic epidermal cells (IDEC) in organotypic models as well as freshly isolated cells from healthy and AD skin...
April 19, 2018: Allergy
https://www.readbyqxmd.com/read/29658453/-braf-v600e-mutation-and-its-clinical-significance-in-children-with-langerhans-cell-histiocytosis
#6
Xue Tang, Xia Guo, Lin-Yong Sun, Yuan Ai, Xue Yang, Jing-Jing Sun, Jian-Rong Wu, Ju Gao
OBJECTIVE: To investigate the clinical significance of BRAF-V600E mutation in children with Langerhans cell histiocytosis (LCH). METHODS: Real-time fluorescence quantitative PCR was used to detect BRAF-V600E mutation in paraffin-embedded tissue samples from 26 children with LCH. A retrospective analysis was performed for the association of BRAF-V600E mutation with clinical features and prognosis of children with LCH. RESULTS: Of the 26 children, 25 received standard chemotherapy, with a 2-year overall survival (OS) rate of 100% and a 2-year event-free survival (EFS) rate of 88%...
April 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29657688/concurrent-mastoid-cellulitis-and-langerhans-cells-histiocytosis-a-challenging-diagnosis
#7
Hui Yan Ong, Liang Chye Goh, Kalimuthu Santhi, Mohamad Mokhtar Sha'ariyah
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder, which commonly arises in the bone and may involve other systems. To date, the diagnosis of temporal bone LCH remains a challenge as it may masquerade as a common ear infection. We report a case of a child who presented to us with persistent bilateral ear discharge for four months and was not responding to treatment. Her condition subsequently worsened, with clinical features and radiological findings suggestive of mastoid cellulitis. Nevertheless, further histopathology study revealed LCH...
March 2018: Oman Medical Journal
https://www.readbyqxmd.com/read/29655588/resident-and-monocyte-derived-langerhans-cells-are-required-for-imiquimod-induced-psoriasis-like-dermatitis-model
#8
Minseok Lee, Sung Hee Kim, Tae-Gyun Kim, Jeyun Park, Jae Won Lee, Min-Geol Lee
BACKGROUND: Langerhans cells (LCs) are dendritic cells that reside in the epidermis and local inflammation results in an increased differentiation of monocyte-derived LCs. Only few studies have investigated on the role of LCs in psoriasis-like dermatitis model, but the results are variable and the exact role of LCs in psoriasis model remains to be elucidated. OBJECTIVE: To explore the functional role of resident (rLCs) and monocyte-derived LCs (mLCs) in imiquimod (IMQ)-induced psoriasis-like inflammation using human Langerin-diphtheria toxin subunit A (huLang-DTA) mice...
April 6, 2018: Journal of Dermatological Science
https://www.readbyqxmd.com/read/29649105/glyteer-soybean-tar-impairs-il-4-stat6-signaling-in-murine-bone-marrow-derived-dendritic-cells-the-basis-of-its-therapeutic-effect-on-atopic-dermatitis
#9
Masaki Takemura, Takeshi Nakahara, Akiko Hashimoto-Hachiya, Masutaka Furue, Gaku Tsuji
Atopic dermatitis (AD) is a common inflammatory skin disease. Recent studies have revealed the involvement of T helper (Th)2 cytokines including Interleukin 4 (IL-4) in the pathogenesis of AD. Since epidermal Langerhans cells (LCs) and dermal myeloid dendritic cells (DCs) produce CCL17 and CCL22 that chemoattract Th2 cells, interfering with CCL17 and CCL22 production from LCs and dermal myeloid DCs may be beneficial in the treatment of AD. To investigate this, we stimulated murine bone marrow-derived DCs (BMDCs) with IL-4...
April 12, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29649018/frequency-of-map2k1-tp53-and-u2af1-mutations-in-braf-mutated-langerhans-cell-histiocytosis-further-characterizing-the-genomic-landscape-of-lch
#10
Lisa M McGinnis, Grant Nybakken, Lisa Ma, Daniel A Arber
Langerhans cell histiocytosis is a proliferative disorder of neoplastic Langerhans cells with activating mutations in the Erk signaling pathway. TP53 and U2AF1 mutations have been implicated in other myelomonocytic malignancies and we hypothesized that mutations in these genes may cosegregate in LCH patients according to BRAF mutation status. Towards this end, we collected cases with a pathologic diagnosis of Langerhans cell histiocytosis from Stanford University Hospital. We analyzed the status of known pathogenic alleles in BRAF, ARAF, TP53, U2AF1, and MAP2K1 on formalin-fixed, paraffin-embedded tissue by direct sequencing...
April 11, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29643005/a-case-report-of-orbital-langerhans-cell-histiocytosis-presenting-as-a-orbital-cellulitis
#11
M Albert-Fort, M González-Candial
CLINICAL CASE: A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell Histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively...
April 8, 2018: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/29642930/neuropeptide-regulation-of-adaptive-immunity-in-the-tibia-fracture-model-of-complex-regional-pain-syndrome
#12
Wen-Wu Li, Tian-Zhi Guo, Xiaoyou Shi, Frank Birklein, Tanja Schlereth, Wade S Kingery, J David Clark
BACKGROUND: Both dysfunctional neuropeptide signaling and immune system activation are characteristic of complex regional pain syndrome (CRPS). Unknown is whether substance P (SP) or calcitonin gene-related peptide (CGRP) support autoantibody production and, consequently, nociceptive sensitization. METHODS: These experiments involved the use of a well-characterized tibia fracture model of CRPS. Mice deficient in SP expression (Tac1-/- ) and CGRP signaling (RAMP1-/- ) were used to probe the neuropeptide dependence of post-fracture sensitization and antibody production...
April 11, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29627328/toxoplasma-gondii-as-a-possible-causative-pathogen-of-type-1-diabetes-mellitus-evidence-from-case-control-and-experimental-studies
#13
Engy Victor Nassief Beshay, Samar A El-Refai, Mohamed A Helwa, Amany Fawzy Atia, Marwa Mohammed Dawoud
Toxoplasma gondii is the causative parasite of an important worldwide disease. This obligate intracellular parasite can infect and replicate inside any nucleated cells including those of pancreas. Insulin is a hormone secreted by the pancreas and is responsible for controlling blood glucose concentration. Deficiency of insulin production accounts for the occurrence of type-1 diabetes mellitus (T1D). Thus, theoretically, toxoplasmosis could play a possible role in the development of T1D. However, the studies on this theory are still insufficient; therefore, this work was designed...
April 5, 2018: Experimental Parasitology
https://www.readbyqxmd.com/read/29625778/-the-epidermal-barrier
#14
R Abdayem, M Haftek
The skin acts as an interface between the body and its surrounding environment. The epidermis, the surface layer of the skin, is chiefly responsible for this interactive protective function. The epidermal barrier may be subdivided into three defensive systems: the photoprotective barrier, the immune barrier, and the physical and chemical barrier of the stratum corneum or horny layer. To protect against harmful ultraviolet radiation, the epidermis has absorption factors such as melanin, produced by melanocytes, and urocanic acid, which is a degradation product of filaggrin...
April 3, 2018: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/29624716/oral-juvenile-xanthogranuloma-in-a-child-clinical-histological-and-immunohistochemical-profile-of-a-rare-entity
#15
Celeste Sánchez-Romero, Ana Isabel Cuenca Arriaga, Oslei Paes de Almeida, Evangelina Gutiérrez Cortés
Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis (non-LCH) affecting normolipemic infants and children most frequently in the first year of life, often showing spontaneous regression within 3 to 6 years. Classic JXG is characterized by a yellowish asymptomatic papule or nodule, often located in the skin of the head, neck and upper trunk. Oral JXG have been reported, but is rare. Histologically JXG is composed mainly by an infiltrate of macrophages with a variable degree of lipidization (foamy macrophages), and (most of the times) scattered Touton type giant cells...
April 6, 2018: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/29624648/cns-langerhans-cell-histiocytosis-common-hematopoietic-origin-for-lch-associated-neurodegeneration-and-mass-lesions
#16
Kenneth L McClain, Jennifer Picarsic, Rikhia Chakraborty, Daniel Zinn, Howard Lin, Harshal Abhyankar, Brooks Scull, Albert Shih, Karen Phaik Har Lim, Olive Eckstein, Joseph Lubega, Tricia L Peters, Walter Olea, Thomas Burke, Nabil Ahmed, M John Hicks, Brandon Tran, Jeremy Jones, Robert Dauser, Michael Jeng, Robert Baiocchi, Deborah Schiff, Stanton Goldman, Kenneth M Heym, Harry Wilson, Benjamin Carcamo, Ashish Kumar, Carlos Rodriguez-Galindo, Nicholas S Whipple, Patrick Campbell, Geoffrey Murdoch, Julia Kofler, Simon Heales, Marian Malone, Randy Woltjer, Joseph F Quinn, Paul Orchard, Michael C Kruer, Ronald Jaffe, Markus G Manz, Sergio A Lira, D Williams Parsons, Miriam Merad, Tsz-Kwong Man, Carl E Allen
BACKGROUND: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH. METHODS: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions...
April 6, 2018: Cancer
https://www.readbyqxmd.com/read/29624481/craniofacial-manifestations-of-systemic-disorders-ct-and-mr-imaging-findings-and-imaging-approach
#17
V Carlota Andreu-Arasa, Margaret N Chapman, Hirofumi Kuno, Akifumi Fujita, Osamu Sakai
Many systemic diseases or conditions can affect the maxillofacial bones; however, they are often overlooked or incidentally found at routine brain or head and neck imaging performed for other reasons. Early identification of some conditions may significantly affect patient care and alter outcomes. Early recognition of nonneoplastic hematologic disorders, such as thalassemia and sickle cell disease, may help initiate earlier treatment and prevent serious complications. The management of neoplastic diseases such as lymphoma, leukemia, or Langerhans cell histiocytosis may be different if diagnosed early, and metastases to the maxillofacial bones may be the first manifestation of an otherwise occult neoplasm...
April 6, 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29622846/improved-serial-sectioning-techniques-for-correlative-light-electron-microscopy-mapping-of-human-langerhans-islets
#18
Sei Saitoh, Nobuhiko Ohno, Yurika Saitoh, Nobuo Terada, Satoshi Shimo, Kaoru Aida, Hideki Fujii, Tetsuro Kobayashi, Shinichi Ohno
Combined analysis of immunostaining for various biological molecules coupled with investigations of ultrastructural features of individual cells is a powerful approach for studies of cellular functions in normal and pathological conditions. However, weak antigenicity of tissues fixed by conventional methods poses a problem for immunoassays. This study introduces a method of correlative light and electron microscopy imaging of the same endocrine cells of compact and diffuse islets from human pancreatic tissue specimens...
February 27, 2018: Acta Histochemica et Cytochemica
https://www.readbyqxmd.com/read/29621879/recurrent-indeterminate-dendritic-cell-tumor-of-the-skin
#19
Jin Woo Joo, Taek Chung, Yoon Ah Cho, Sang Kyum Kim
Indeterminate dendritic cell tumor (IDCT) is a dendritic cell tumor that displays histologic features similar to those of Langerhans cells. The origin of the indeterminate cells may represent precursors of Langerhans cells or skin dendritic cells. IDCT is extremely rare, and tumor progression and predictive factors are not well known. Here, we report a case of a 61-year-old man who presented with a papule on his back and was finally diagnosed with IDCT based on histology and immunohistochemistry. The tumor recurred months after surgical excision...
April 5, 2018: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/29618940/langerhans-cell-histiocytosis-with-hemorrhagic-uveitis-and-exudative-retinal-detachment
#20
Ranju Kharel Sitaula, Anadi Khatri
Bilateral intraocular involvement in Langerhans cell histiocytosis (LCH) is uncommon. A 15-year-old boy presented with painless decreased vision in right and painful left red eye of 2 weeks duration. Visual acuity was 20/500 and 20/200, respectively. A fixed dilated pupil with exudative retinal detachment was present in the right eye and hemorrhagic iris nodules with hyphema and hypopyon were seen in the left eye. Intraocular pressure was 12 and 31 mmHg, respectively, in each eye. Ocular symptoms were preceded by fever with multiple skin rashes, subcutaneous nodules, and lymph node enlargement...
2018: International Medical Case Reports Journal
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