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Erdheim–Chester disease

Thoracic, abdominal and musculoskeletal involvement in Erdheim-Chester disease: CT, MR and PET imaging findings

Thoracic, abdominal and musculoskeletal involvement in Erdheim-Chester disease: CT, MR and PET imaging findings

... Background Erdheim-Chester disease (ECD) is a rare, non- Langerhans cell histiocytosis with characteristic radiological and histological features. This entity is defined by a mononu- clear infiltrate ...

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Multimodal Chorioretinal Imaging in Erdheim-Chester Disease

<p>Multimodal Chorioretinal Imaging in Erdheim-Chester Disease</p>

... Erdheim-Chester disease (ECD) is a rare, multisystemic disease caused by the accumulation and in fi ltration of non-Langerhans foamy histiocytes in different body sites; about 700 cases have ...

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Symptomatic empty sella syndrome: an unusual manifestation of Erdheim–Chester disease

Symptomatic empty sella syndrome: an unusual manifestation of Erdheim–Chester disease

... ErdheimChester disease (ECD) remains a challenging case to diagnose and treat. ECD should be considered in patients with hypogonadotropic hypogonadism and hypopituitarism on a background of ...

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Erdheim Chester disease in a patient with Burkitt lymphoma: a case report and review of literature

Erdheim Chester disease in a patient with Burkitt lymphoma: a case report and review of literature

... Background: Erdheim Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis characterized by widespread tissue infiltration by CD68-positive, CD1a-negative foamy ...

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Case Report Erdheim-Chester Disease with Involvement of Axial Skeleton: A Rare Presentation of Rare Disease

Case Report Erdheim-Chester Disease with Involvement of Axial Skeleton: A Rare Presentation of Rare Disease

... Erdheim-Chester disease (ECD) is a non-Langerhans cell systemic histiocytosis with an unknown etiology and ...as Erdheim-Chester disease (ECD) on the basis of clinical , ...

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Fever of unknown origin as the first manifestation of Erdheim Chester disease

Fever of unknown origin as the first manifestation of Erdheim Chester disease

... Erdheim-Chester disease (ECD) is an uncom- mon non-Langerhans cell histiocytosis that af- fects multiple body systems and can present clinically in a myriad of ...

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Diffuse reduction of cerebral grey matter volumes in Erdheim-Chester disease

Diffuse reduction of cerebral grey matter volumes in Erdheim-Chester disease

... Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis reported in approximately 550 cases since its initial description in ...multisystem disease characterized by ...

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Unusual manifestation of Erdheim-Chester disease

Unusual manifestation of Erdheim-Chester disease

... Due to ongoing vomiting and weight loss, a gastroscopy was performed with no significant pathology. Computer tomography (CT) of the chest showed peri-aortic tissue infiltration creating the appearance of “coated aorta” ...

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Erdheim-Chester Disease: a comprehensive review of the literature

Erdheim-Chester Disease: a comprehensive review of the literature

... this disease is still poorly ...the disease as published in former case reports and literature ...singular disease entity in the medical litera- ture, baring specific diagnostic ...

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BRAF V600E mutation in Juvenile Xanthogranuloma family neoplasms of the central nervous system (CNS-JXG): a revised diagnostic algorithm to include pediatric Erdheim-Chester disease

BRAF V600E mutation in Juvenile Xanthogranuloma family neoplasms of the central nervous system (CNS-JXG): a revised diagnostic algorithm to include pediatric Erdheim-Chester disease

... and Erdheim Chester disease ...CNS disease ( BRAF V600E: 80% vs WT: 20%) and systemic disease ( BRAF V600E: 40% vs WT: ...aggressive disease presentation including pediatric ...

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Clinical and positron emission tomography responses to long-term high-dose interferon-α treatment among patients with Erdheim–Chester disease

Clinical and positron emission tomography responses to long-term high-dose interferon-α treatment among patients with Erdheim–Chester disease

... Patients with ECD have been reported to have elevated levels of IFN- α , IL-12, chemokine ligand 18 (CCL18) and monocyte chemotactic protein-1 but decreased levels of IL-4 and IL-7; however, a previous study failed to ...

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Clinical considerations and key issues in the management of patients with Erdheim-Chester Disease: a seven case series

Clinical considerations and key issues in the management of patients with Erdheim-Chester Disease: a seven case series

... heart and great vessels [12-15], lungs [16-18], kidneys and retroperitoneum [19-23], adrenal glands [24], skin [25-27], gastrointestinal tract [28-31], breast [32-34], skeletal muscle [32,35], thyroid and testis [36]. ...

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Erdheim Chester Disease of the Central Nervous System: New Manifestations of a Rare Disease

Erdheim Chester Disease of the Central Nervous System: New Manifestations of a Rare Disease

... tients in our study who demonstrated linear thickening of the pituitary stalk. In the fourth patient, the disease involved the sella and extended into the cavernous sinuses. Johnson et al 13 described a similar ...

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Clinical Reasoning: Accumulating endocrinopathies in a car salesman

Clinical Reasoning: Accumulating endocrinopathies in a car salesman

... symptoms. Erdheim-Chester disease has a chronic course and may also cause a chronic pituitary dysfunction but the absence of pain caused by bone involvement makes this diagnosis less ...

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Effective BRAF inhibitor vemurafenib therapy in a 2-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim&ndash;Chester disease

Effective BRAF inhibitor vemurafenib therapy in a 2-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim&ndash;Chester disease

... Abstract: ErdheimChester disease (ECD) is a rare histiocytic disorder, characterized by the xanthomatous infiltration of tissues by CD68-positive and CD1a-/CD100-negative foamy ...

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Clinical Reasoning: A 52-year-old man with diplopia and ataxia

Clinical Reasoning: A 52-year-old man with diplopia and ataxia

... Intrinsic brainstem glioma is rare in adults but may mimic brainstem encephalitis. Primary CNS lymphoma, lymphomatosis cerebri, intravascular lymphoma, and even primary CNS germ cell neoplasms might rarely demonstrate ...

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Schnitzler’s syndrome: lessons from 281 cases

Schnitzler’s syndrome: lessons from 281 cases

... supported by different lines of evidence. First, several cases have been reported in which a M-protein became detect- able several years after the onset of symptoms. In a Norwegian patient, for example, this was 13 years ...

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The city and its canal: producing a place marketing pilot plan for Chester

The city and its canal: producing a place marketing pilot plan for Chester

... The PMPP was produced in the context of the INTERREG IIIc CultMark project (Cultural Heritage, Local Identity and Place Marketing for Sustainable Development) that was in operation in five European places from 2004 till ...

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Annual report for the year 1922

Annual report for the year 1922

... "Mellifont," High Street, Sandy Lord, Chester.. "Handroyd," 6 Franklin Street, Lord, Raymond.[r] ...

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Laudianism in the Diocese of Chester: Revisiting the Episcopate of John Bridgeman

Laudianism in the Diocese of Chester: Revisiting the Episcopate of John Bridgeman

... in Chester, who had been at the centre of a sabbatarian storm in Chester in the early 1630s into which Bridgeman had been ...in Chester Cathedral, Ley (as sub-dean of the Cathedral) wrote to ...

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