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Histiocytosis Langerhans-Cell
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1. Ocular adnexal Langerhans cell histiocytosis clinical features and management.
Match Strength: 10.940

PURPOSE: To review the clinical features, investigations, management, and outcomes of Langerhans cell histiocytosis (LCH) with ocular adnexal involvement. MATERIALS AND METHODS: Retrospective, non-comparative, chart review of 30 patients with LCH involving the ocular adnexa treated at 6 major Australian hospitals. Clinical features, imaging findings, treatment, local and distant recurrence and outcome were evaluated. RESULTS: Twenty-four patients (80%) were male with a mean age of presentation of 9.5 years. Females presented at an earlier age (mean age 4.1 years) with more severe involvement. ... Read More »
» Published in Orbit. 2006 Sep;25(3):169-77.

2. Idiopathic central diabetes insipidus in children and young adults is commonly associated with vasopressin-cell antibodies and markers of autoimmunity.
Match Strength: 6.678

OBJECTIVES: Autoimmune targeting of hypothalamic-neurohypophyseal structures in children and young adults with posterior pituitary and anterior pituitary dysfunction, as well as pituitary stalk involvement, are not yet completely understood. DESIGN: We aimed to (1) evaluate the presence of circulating vasopressin-cell autoantibodies (AVPc-Abs) in young patients with central diabetes insipidus (CDI), (2) detect organ-specific autoantibodies as markers of autoimmunity, and (3) define the relationship between immune markers and neuroimaging findings. PATIENTS: Twenty patients were evaluated at a ... Read More »
» Published in Clin Endocrinol (Oxf). 2006 Oct;65(4):470-8.

3. Variability in the efficacy of interferon-alpha in Erdheim-Chester disease by patient and site of involvement: results in eight patients.
Match Strength: 5.838

OBJECTIVE: Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown origin, characterized by infiltration of tissues by spumous histiocytes. ECD features heterogeneous systemic manifestations, and the general prognosis remains poor despite various treatment options. METHODS: We treated 8 patients with multisystemic ECD with subcutaneous interferon-alpha (IFNalpha) at a dosage of 3-9 x 10(6) units 3 times weekly, for a median duration of 23 months (range 1-46 months). RESULTS: Treatment was generally well tolerated, and side effects remained limited to fever ... Read More »
» Published in Arthritis Rheum. 2006 Oct;54(10):3330-6.

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