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)

After reviewing the syndrome of histiocytosis X, the special problems of an attack on the eye are discussed with regard to clinical, histological and radiological findings. By means of two cases, all aspects important for the diagnosis and treatment of histiocytosis X are discussed with reference to the literature. A histologically verified histiocytosis X in the area of the orbita is described in an almost 2 year old girl and another girl aged 7 years and 3 months. In both cases, the disease began with an edema of the upper eyelid and swelling of the lateral orbital margin. The second case showed in addition the clinical symptoms of a dacryoadenitis. The X-ray pictures of the skull revealed a large triangular osseous lesion in the area of the orbital margins. Blood chemistry, hematological tests and the bonemarrow puncture did not yield any pathological findings. The diagnosis of histiozytosis X could be made only by a biopsy from the tumor area. The swelling in the area of the eyelids and the lateral orbital margin disappeared under cytostatic therapy with Vincristin and Prednison.
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PMID:[Histiocytosis-X of the orbit (author's transl)]. 108 43

Diseases of the posterior compartment and the orbit are characterised by histological findings, most of which can be reproduced clinically. Examples are the examination of calcifications in retinoblastoma by ultrasonography. In the present review, histological findings of tumour and other diseases of the posterior ocular compartment and the orbit are presented and correlated with the clinical pictures and imaging techniques: uveal melanoma, choroidal nevus, choroidal metastases, choroidal hemangioma, retinoblastoma, Coat's disease, sympathetic ophthalmia, pleomorphic adenoma (benign mixed tumour) of the lacrimal gland, dacryoadenitis, lymphoma, rhabdomyosarcoma, Langerhans cell histiocytosis, orbital metastases, and phthisical eyes. Histopathology is usually the gold standard for a definitive diagnosis. It is very important for residents and those in training to become familiar with clinico-pathological correlations as these provide insight in pathophysiological processes. Regarding ophthalmic surgery, ophthalmic pathology offers the possibility to study wound healing and complications. A close collaboration between clinicians and ocular pathologists allows for an optimised processing of the submitted tissue and diagnosis. Thus, pre- and postoperative care can also be improved. This outstanding knowledge that ophthalmologists have gained over the last decades and beyond, should be preserved and passed on to the next generations in order to maintain a high standard in ophthalmological care.
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PMID:[Clinico-pathological correlations: posterior compartment of the eye and orbit]. 2283 34