Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X encompasses three syndromes characterized by the idiopathic proliferation of histiocytes: eosinophilic granuloma, Hand-Schuller-Christian syndrome, and Letterer-Siwe disease. At the Mayo Clinic between 1926 and 1978, 22 patients with histiocytosis X had involvement of the ear or temporal bone. These patients comprised 15% of all patients with histiocytosis X seen during that period. The ages at onset of the disease ranged from 2 months to 49 years. The most frequent otologic symptom was aural discharge (15 patients), followed in frequency by swelling in the temporal region (11 patients), vertigo (6 patients), and deafness (5 patients). Clinical findings included otitis media (13 patients), otitis externa with or without granulation tissue (10 patients), and osteolytic lesions of the temporal bone (9 patients). A high index of suspicion is required to recognize the otologic manifestations of histiocytosis X for two reasons: the systemic manifestations of the disease are often so dramatic that the ear findings are overlooked, and the otologic findings of histiocytosis X can mimic more common diseases, including simple otitis externa, aural polyps, acute mastoiditis, chronic otitis media, and metastatic lesions.
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PMID:Histiocytosis X of the ear and temporal bone: review of 22 cases. 31 4

This 20-year retrospective review identifies 35 pediatric patients with aural polyps in an attempt to assess for clinical predictors of significant otopathologic conditions. Chronic otitis media (43%), cholesteatoma (29%), and retained tympanostomy tubes (23%) were the common causes. Unusual causes included mycobacterial infection and Langerhans' cell histiocytosis. Multivariate analysis revealed the co-occurrence of conductive hearing loss at presentation to be a significant clinical predictor (P = .03) of cholesteatoma; the histopathologic finding of keratin-induced giant cell reaction was nonspecific in this respect. Cholesteatoma was also prevalent in recurrent polyp cases, suggesting the need for prolonged follow-up in those children whose initial clinicopathologic evaluation does not yield a definitive diagnosis.
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PMID:The cause of aural polyps in children. 849 99

Otorrhea varies in appearance and can be accompanied by a multitude of symptoms. Recognition of what various combinations indicate--whether otitis externa, chronic otitis media, Langerhans' cell histiocytosis, or basal or squamous cell carcinoma--is essential. Physicians must also be familiar with the tests appropriate for confirming otorrhea's cause. Once proper diagnosis is made, effective treatment can follow.
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PMID:Which culprit is causing your patient's otorrhea? 974 3

Langerhans cell histiocytosis (LCH) is a rare disease ranging from a benign to a rapidly fatal condition affecting young children predominantly, and is characterized by an abnormal clonal proliferation of Langerhans cells. We report a case of a 3-year-old child presenting with a 1-year history of otorrhea and otorrhagia followed by a 6-month history of postauricular swelling in the right ear. Imaging demonstrated a large mass of organized tissue. A biopsy was conducted, and the diagnosis of LCH was confirmed by histopathological and immunohistochemical examination. The child was treated with a 12-month course of vinblastine chemotherapy with prednisolone. No clinical evidence of recurrence was noticed after 3 years of follow-up. This rare case highlights the importance for otolaryngologists to keep LCH in mind for differential diagnosis in very young patients with symptoms and signs suggestive of acute mastoiditis or chronic otitis media.
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PMID:A rare disorder mimics otitis media: Langerhans cell histiocytosis of the temporal bone in a child with interstitial pulmonary fibrosis. 2513 21