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Chester Erdheim disease

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https://www.readbyqxmd.com/read/28099231/vemurafenib-braf-inhibitor-therapy-for-orbital-erdheim-chester-disease
#1
Adit Gupta, Amir Yeganeh, Daniel Rootman, Robert Goldberg
Erdheim-Chester disease is a rare xanthogranulomatous systemic disease, which involves the orbit in some cases. Through this case report, the authors review the treatment modalities used in orbital Erdheim-Chester disease and explore a newer modality of treatment. Cases of orbital Erdheim-Chester disease were identified in the literature utilizing a PubMed search and all the treatment modalities were reviewed. The response to treatment of orbital Erdheim-Chester disease has been poor with the various medical and surgical treatment modalities used in the past...
January 17, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28095017/spectrum-of-xanthogranulomatous-processes-in-the-abdomen-and-pelvis-a-pictorial-review-of-infectious-inflammatory-and-proliferative-responses
#2
Kelsey S Bourm, Christine O Menias, Kamran Ali, Kinan Alhalabi, Khaled M Elsayes
OBJECTIVE: Xanthogranulomatous (XG) processes are rare inflammatory conditions with the characteristic pathologic feature of lipid-laden macrophages or histiocyte cells. Imaging findings are nonspecific and can simulate aggressive neoplastic processes. XG processes can be caused by infection, inflammation, histolytic process, or an inherited lysosomal disorder. XG infectious processes are mainly seen in cholecystitis and pyelonephritis, but several other organs can also be involved. Histiocytic processes can be divided into Langerhans and non-Langerhans cell histiocytosis...
January 17, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28080175/combination-of-adalimumab-with-lower-dose-of-methylprednisolone-in-erdheim-chester-disease-with-systemic-involvement
#3
Xin Jia, Xiaoxuan Ning, Qingxian Bai, Nan Leng, Shengjun Wang, Xiaoming Wang, Shoujing Yang
No abstract text is available yet for this article.
January 12, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/28078666/pediatric-erdheim-chester-disease-with-aggressive-skin-manifestations
#4
H-H Su, W Wu, Y Guo, H-D Chen, S-J Shan
Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, is uncommon and characterized by the accumulation of CD68+/CD1a- foamy histiocytes. It is extremely rare in children. The skin lesions of pediatric ECD was not systemically described before. We report a case of ECD in a 3.5 year old Chinese boy. The patient present with generalized skin and bone involvement for 3 years duration. Marked generalized annular maculopapular lesions with central atrophy which differed from previously reported adult xanthoma-like papules or periorbital xanthelasma-like lesions...
January 12, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28051224/erdheim-chester-disease-and-radiobiphosphonates-bone-scan-from-a-simple-peripheral-form-to-a-rare-axial-location-a-case-report
#5
Salah Oueriagli Nabih
The Erdheim-Chester disease is a rare form of non Langherans histiocytosis. It can be expressed by a multi systemic disease interesting: bone, lung, heart, brain, retro peritoneum, skin and retro-orbital tissue. The diagnosis is established under the radiological aspect, bone scan uptake and immunohistological profile. The bone lesions are almost constant and rather characteristic of bone scintigraphy. We related the case of a young female patient of 26 years old with Erdheim-Chester bone disease. Diagnosis was revealed by diabetes insipidus (nerve damage) and diffuses bones pain...
June 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27995058/erdheim-chester-disease-moving-away-from-the-orphan-diseases-a-case-report
#6
Jessica M Stempel, Jean G Bustamante Alvarez, Andres Mora Carpio, Varun Mittal, Claudia Dourado
With approximately 750 cases reported, Erdheim-Chester disease is an exceedingly rare histiocyte cell disorder. Affected sites typically include long bones, large vessels and central nervous system. However, cutaneous and pulmonary involvement can also occur. The diagnosis is ascertained by identification of foamy histiocytes positive for CD68, CD163, and factor XIIIa on immunoperoxidase staining. Recently published literature have described an association between Erdheim-Chester disease and BRAF V600E mutation...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27940476/trametinib-following-disease-reactivation-under-dabrafenib-in-erdheim-chester-disease-with-both-braf-and-kras-mutations
#7
Thierry M Nordmann, Freimut D Juengling, Mike Recher, Christoph T Berger, Daniel Kalbermatten, Andreas Wicki, Aino Paasinen-Sohns, Gieri Cathomas, Alexandar Tzankov, Thomas Daikeler
Major advances have been made in understanding the pathogenesis of Erdheim-Chester disease (ECD) leading to novel treatment strategies. Targeted therapies such as BRAF inhibition have shown a significant impact on disease management, emphasizing the importance of the activated mitogen-associated protein kinase (MAPK) pathway in this disease. However, incomplete responsiveness, potentially limiting side effects and the occurrence of treatment resistance to BRAF inhibition observed in other BRAF mutant malignancies imply the importance of therapeutic strategies beyond BRAF inhibition...
December 9, 2016: Blood
https://www.readbyqxmd.com/read/27900869/-evaluation-of-five-years-of-treatment-of-erdheim-chester-disease-with-anakinra-case-report-and-overview-of-literature
#8
Zdeněk Adam, Hana Petrášová, Zdeněk Řehák, Renata Koukalová, Marta Krejčí, Luděk Pour, Eva Vetešníková, Aleš Čermák, Sabina Ševčíková, Petr Szturz, Zdeněk Král, Jiří Mayer
: Erdheim-Chester disease is a histiocytic neoplasm of diseases from the group of non-Langerhans-cell histiocytoses, formed by infiltrates of foamy histiocytes. These pathological histiocytes produce pro-inflammatory cytokines. Therefore Erdheim-Chester disease is called inflammatory histiocytary neoplasm. The disease is accompanied by clinical symptoms of systemic inflammatory response, i.e. B symptoms. Imaging examinations detect typical osteosclerotic changes affecting diaphyses and metaphyses of the lower long bones and fibrotic changes which affect the aorta wall and the vessels leading from it...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27898473/histopathological-and-clinical-findings-in-cutaneous-manifestation-of-erdheim-chester-disease-and-langerhans-cell-histiocytosis-overlap-syndrome-associated-with-the-brafv600e-mutation
#9
Julia Liersch, J Andrew Carlson, Jörg Schaller
The overlap of Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) is more common than it was generally accepted. Both diseases seem to be linked by a mutation in oncogenic BRAFV600E, probably an early event which occurs in bone marrow progenitor cells. In this article are described the clinical and histological findings in 2 cases of ECD-LCH overlap syndrome bearing the BRAFV600E mutation in both ECD and LCH lesions in bone and skin. In one case, lesions of ECD and LCH were situated directly site-to-site in the same bone section leading to the assumption of a common myeloid precursor cell for these diseases...
November 23, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27889325/-langerhans-cell-histiocytosis-and-erdheim-chester-disease-a-continuity
#10
S Parreau, J Haroche, I Pommepuy, J F Emile, J C Bourras, F Archambeaud
INTRODUCTION: Erdheim-Chester disease and langerhans cell histiocytosis are two rare diseases separate on clinical, radiological and histological aspects. However, several cases involving both entities have been described. OBSERVATION: A 70-year-old man had a central diabetes insipidus, xanthelasmas, retroperitoneal fibrosis and osteosclerosis of the legs suggestive of Erdheim-Chester disease. Bone biopsy showed langerhans cell histiocytosis CD1a positive with the presence of BRAF V600E mutation...
November 23, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27863426/quantification-of-tumor-derived-cell-free-dna-cfdna-by-digital-pcr-digpcr-in-cerebrospinal-fluid-of-patients-with-brafv600-mutated-malignancies
#11
Parisa Momtaz, Elena Pentsova, Omar Abdel-Wahab, Eli Diamond, David Hyman, Taha Merghoub, Daoqi You, Billel Gasmi, Agnes Viale, Paul B Chapman
Tumor-derived cell free DNA (cfDNA) can be detected in plasma. We hypothesized that mutated BRAF V600 cfDNA could be quantified in the cerebrospinal fluid (CSF) of patients with central nervous system (CNS) metastases. We collected CSF from patients with BRAF V600E or K-mutated melanoma (N=8) or BRAF V600E mutated Erdheim-Chester Disease (ECD) (N=3) with suspected central nervous system (CNS) involvement on the basis of neurological symptoms (10/11), MRI imaging (8/11), or both. Tumor-derived cfDNA was quantified by digital PCR in the CSF of 6/11 patients (range from 0...
November 16, 2016: Oncotarget
https://www.readbyqxmd.com/read/27861332/systemic-vasculitis-associated-with-vemurafenib-treatment-case-report-and-literature-review
#12
Adrien Mirouse, Léa Savey, Fanny Domont, Cloé Comarmond, Stéphane Barete, Emmanuelle Plaisier, Philippe Rouvier, Patrice Cacoub, David Saadoun
RATIONALE: Vemurafenib, an inhibitor of mutated B-rapidly accelerated fibrosarcoma, is frequently used in the treatment of melanoma and Erdheim-Chester disease (ECD) patients. Inflammatory adverse effects have been increasingly reported after vemurafenib treatment. PATIENT CONCERNS AND DIAGNOSE: We report 6 cases of vemurafenib-associated vasculitis, of whom a personal case of a 75-year-old man with history of ECD who developed purpura and rapidly progressive pauci-immune glomerulonephritis during treatment with vemurafenib...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27833932/targeted-treatment-of-brainstem-neurohistiocytosis-guided-by-urinary-cell-free-dna
#13
David Hunt, Paul Milne, Peter Fernandes, Venetia Bigley, Matthew Collin
OBJECTIVE: To identify a treatment-responsive BRAF(V600E) mutation in brainstem neurohistiocytosis, where no lesional tissue was readily obtainable, using a cell-free DNA approach. METHODS: Cell-free DNA was extracted from urine and allele-specific PCR for the BRAF(V600E) mutation was performed. Response to conventional treatment (corticosteroids and interferon) and targeted treatment with a BRAF inhibitor was assessed by clinical evaluation, gadolinium-enhanced MRI brain scan, and serial testing of urinary cell-free DNA for mutant alleles...
January 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27793652/erdheim-chester-disease-presenting-as-a-solid-renal-mass
#14
Matthew B Clements, Jacques Farhi, Noah S Schenkman
Erdheim-Chester disease (ECD) is a rare, systemic histiocytic disorder, usually presenting due to bone manifestations. The most commonly seen urologic manifestation is retroperitoneal fibrosis, leading to ureteral obstruction. We describe a patient with a solid right renal mass, initially concerning for renal cell carcinoma, in addition to diffuse retroperitoneal fibrosis and a chronically obstructed left kidney. The renal mass and retroperitoneal fibrosis are thought to be caused by ECD based on imaging findings and biopsy results...
October 25, 2016: Urology
https://www.readbyqxmd.com/read/27759649/erdheim-chester-disease-ecd-case-report-clinical-and-basic-investigations-and-review-of-literature
#15
Mohammad Adawi, Bishara Bisharat, Abdalla Bowirrat
BACKGROUND: Erdheim-Chester disease (ECD) is an uncommon aggressive, multisystem form of non-Langerhans' cell histocytosis, which was firstly reported by Jakob Erdheim and William chester in 1930. The disease pathological features encompass an aberrant multiplication, overproduction and accumulation of white blood cells called histiocytes within multiple tissues and organs. Herein, we present a case of ECD owing to the rarity of this disease (roughly 550 cases have been described in the literature to date)...
October 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27752407/a-case-of-incidentally-diagnosed-erdheim-chester-disease
#16
Atman A Dave, Susan E Gutschow, Christopher M Walker
Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytosis that may be clonal and inflammatory in origin. The hallmark of the disease is infiltration of various organ systems by CD68+/CD1a- histiocytes containing foamy lipid-laden inclusions. The manifestations and course of the disease are variable and depend on the organ systems that are affected. Patients may be asymptomatic or may develop life-threatening complications, including myocardial infarction. The most common clinical manifestation is lower extremity bone pain...
September 13, 2016: Curēus
https://www.readbyqxmd.com/read/27739223/an-unusual-presentation-of-erdheim-chester-disease
#17
Sang Gyu Park, Dong Hoon Baek, Kyu Min Lee, Seo Young Oh, Do Youn Park, Sangjeong Ahn, Nari Shin, Geun Am Song
No abstract text is available yet for this article.
October 13, 2016: Journal of Digestive Diseases
https://www.readbyqxmd.com/read/27702933/erdheim-chester-disease-25-year-history-with-early-cns-involvement
#18
C M Rice, C A Hall, P McCoubrie, S A Renowden, N Cohen, N J Scolding
We report a case of Erdheim-Chester disease (ECD) with a 25-year history following initial presentation with diabetes insipidus and brainstem involvement. The exceptionally long history is particularly notable, given that ECD is a life-threatening disorder and there is a recognised association between central nervous system involvement and poor outcome. The case is a timely reminder of the presenting features of the condition, given the emergence of potential new treatment options.
October 4, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27663675/cardiac-involvement-in-erdheim-chester-disease-an-mri-study
#19
Davide Gianfreda, Alessandro A Palumbo, Enrica Rossi, Lorenzo Buttarelli, Gaia Manari, Chiara Martini, Massimo De Filippo, Augusto Vaglio
No abstract text is available yet for this article.
September 23, 2016: Blood
https://www.readbyqxmd.com/read/27648170/unusual-presentation-of-erdheim-chester-disease-in-a-child-with-acute-lymphoblastic-leukemia
#20
Archana George Vallonthaiel, Asit Ranjan Mridha, Shivanand Gamanagatti, Manisha Jana, Mehar Chand Sharma, Shah Alam Khan, Sameer Bakhshi
Erdheim-Chester disease (ECD) is an uncommon, non-familial, non-Langerhans cell histiocytosis, which involves skeletal system and soft tissue usually in middle aged and elderly patients. The characteristic radiologic features include bilateral, symmetric cortical osteosclerosis of the diaphyseal and metaphyseal parts of the long bones, or bilateral symmetrically abnormal intense (99m)Technetium labelling of the metaphyseal-diaphyseal region of the long bones, and computed tomography scan findings of "coated aorta" or "hairy kidneys"...
August 28, 2016: World Journal of Radiology
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