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

Report of a 21-year old male with biopsy-proved (primary) isolated Histiocytosis X (eosinophilic granuloma) localized in the lungs. The disease prefers young males and leads to a non-characteristical fibrosis of the lung in cases of chronical course. Lung biopsy is the only certain method for diagnosis. Morphology, differential-diagnosis and aetiology of this rare disease are also considered.
 ...
PMID:[Histiocytosis X (eosinophilic granulomatosis) of the lung (author's transl)]. 30 May 32

The current concepts of histiocytosis X are discussed, and the literature concering this rare disease of obscure aetiology reviewed. Two case reports of histiocytosis X presenting in similar sites in young children of similar ages, but with markedly different courses, are presented. The importance of early recognition of the disease, particularly by the dental surgeon who may see the first oral signs, is stressed. Attention is drawn to the danger of misdiagnosing the oral signs of histiocytosis X, particularly as periodontal disease. The possible aetiologies are examined and current treatment regimes discussed.
 ...
PMID:Histiocytosis X: current concepts and a report of two cases. 31 12

This is a report on 2 adult patients suffering from pulmonary histiocytosis X. The aetiology and the diagnostic and therapeutic approach are discussed on the basis of a review of the literature. This is a rare disease that is triggered by a virus and immunologically conditioned, the disposition being genetically transferred. It is characterised by cells known as histiocytosis X cells with typical X bodies and immunocytochemically identifiable S 100 antigen. It will usually be necessary to perform an open biopsy of the lung to determine the histology of histiocytosis X. Roentgenologically pathognomonic signs are in particular ring-shaped structures of up to 5 mm diameter with a marginal edge. Lung function analysis revealing hypoxaemia after stress and, less significantly, diffusion capacity and vital capacity, are also among the most sensitive data pointing to histiocytosis X. Indication for treating the patients, who usually do not display prominent signs and symptoms, should be discrete because spontaneous remissions occur very frequently. If the patients display relevant signs and symptoms, corticosteroid long-term treatment over 12 months with 0.5-1.0 mg/kg body weight per day is recommended employing a slowly and progressively reduced dosage schedule. Chemotherapeutic drugs or thymus extracts are administered in a few rare instances.
 ...
PMID:[Pulmonary histiocytosis X]. 194 59

Histiocytosis-X is a difficult disease on which to perform clinical studies. It is a rare disease, with great clinical heterogeneity. The natural history is characterized by periods of spontaneous disease stabilization, remission, and exacerbation. Clinical care is given by several diverse types of physicians. Several suggestions are made for the optimal design and performance of clinical studies aimed at evaluating potentially new therapies, including the proper identification and use of controls. In addition, a staging system is described, and a comprehensive scoring system is given. This scoring system will allow accurate determination of disease activity, and permit analysis of the clinical effectiveness of any new treatment.
 ...
PMID:Evaluating the role of therapy in histiocytosis-X. Clinical studies, staging, and scoring. 349 31

Histiocytosis X is a relatively rare disease presenting as 3 clinical syndromes: eosinophilic granuloma, Hand-Schuller-Christian disease, and Abt-Letterer-Siwe disease. Although the clinical expressions of the disease differ, the underlying histopathology appears to be the same. Since the disease frequently involves and not infrequently presents in the head and neck region, it is particularly relevant to the otolaryngologist. We have recently treated two young children presenting with bilateral otorrhea. Although histiocytosis X was entertained early in the evaluation of these patients, the diagnosis was difficult and prolonged in both cases. One case ultimately required electron microscopic study to confirm the diagnosis. Important aspects of the diagnosis are reviewed. Treatment of histiocytosis X includes surgery, radiation therapy and chemotherapy. These treatment strategies are discussed, with particular emphasis on the management of the ear involvement.
 ...
PMID:Histiocytosis X: otologic presentations. 387 41

Langerhans' cell granulomatosis is a rare disease whose incidence on adults is valued about one case out of 3.000.000. The multifocal form is rarer than the single eosinophilic granuloma of the bone. It mainly affects males during childhood and it is usually localized in the long bones, hands, feet, and in the skull. We present the case of a young woman affected by multifocal Langerhans cell granulomatosis with bones and skin involved. Unusual in the presented clinical case is the age in which the disease come out. There is the highest incidence of multifocal lesions during childhood, while the monofocal kind is more typical of the years of adult age. Bone localization in the sternum is also quite unusual. In fact it is impossible to find reports of it in recent literature. As the patient refused to undergo chemotherapy she was treated with radiant therapy as for as bone localization concerned and with mecloretamin for local administration on skin lesion. Eleven months after the diagnosis we have not noticed the appearance of new lesions.
 ...
PMID:[Multifocal Langerhans cell granulomatosis. Report of a clinical case]. 793 61

Hospital records of seventeen patients (11 males & 6 females) with Langerhans cell histiocytosis, confirmed by electron microscopic demonstration of Birbeck granules, were studied retrospectively from October 1982 to October 1992 at Taichung Veterans General Hospital. The ages at presentation ranged from 5 months to 17 years (a median of 6 years). The clinical features were protean and included fever, pain, bony lesions, lung lesions, abnormal dentition, diabetes insipidus, oral ulcer, otorrhea, dermatitis, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. Skull and femur were the most common sites of bony lesions. The main therapeutic modalities were excision, radiotherapy, and chemotherapy with vincristine and prednisolone. The young age at presentation, several involved organ systems, presence of organ dysfunction, and clinical diagnosis with Letterer-Siwe disease were poor prognostic factors. Although Langerhans cell histiocytosis is not a rare disease, the cause is still unknown. It needs further research to disclose the mystery.
 ...
PMID:Langerhans cell histiocytosis: a 10-year review. 794 24

Congenital histiocytosis X involving multiple organs is a rare disease that causes rapid mortality in intrauterine and neonatal life. The diagnosis of histiocytosis X (Letterer-Siwe disease) should be considered in a neonate with vesiculated crusting skin lesions. We present clinical, radiographic and histopathological findings in a neonate with congenital histiocytosis who died of respiratory failure due to diffuse infiltration of lungs with histiocytic cells.
 ...
PMID:Congenital histiocytosis X. 833 3

Histiocytosis X, or Langerhans cell histiocytosis (LCH), is a rare disease, with an estimated incidence of 1/200,000 per year in children under 15 years of age. It has a wide clinical spectrum, from single bone involvement (eosinophilic granuloma) to multisystemic disease with organ failure. The treatment of LCH is still controversial. While single system disease may spontaneously recover or requires minimal treatment, some multisystemic disease with organ dysfunction may resist systemic chemotherapy. The mortality rate in this latter subgroup of patients is relatively high and reaches 40% of the cases. A rational approach to the treatment of LCH is based on a better understanding of clinical and pathophysiological features. A prospective multicentre trial including scientific research should be planned, in order to determine the optimal treatment of LCH and to ameliorate the prognosis of the severe unresponsive form of the disease.
 ...
PMID:[Langerhans cell histiocytosis]. 874 30

A 28-year-old woman was admitted to our hospital with the chief complaint of progressive gingival swelling with easy bleeding and loosening of teeth for about two years. The liver and spleen were not enlarged and there were no lymphadenopathy. The hemogram was normal. The skull X-ray showed floating teeth. CT scan of face showed destructive bony lesions over maxilla and left mandible with adjacent soft tissue swelling. Pathologic examination of the gingiva revealed that the oral mucosal tissue was heavily infiltrated with histiocyte-like Langerhans' cells. The Langerhans' cells showed positive immunostain for S-100 protein. Under electron microscope, ultrastructure of the Langerhans' cells revealed typical intracytoplasmic tennnis racquet-shaped structure with a central zipper-like striation (Birbeck's granules). Langerhans' cell histiocytosis was diagnosed. She received rdiotherapy with a total dose of 1,000 cGy in fractions and oral chemotherapeutic drugs. Painful gingival swelling subsided gradually. She was followed at our OPD for the past 10 months and there was no evidence of local recurrence. Langerhans' cell histiocytosis is a rare disease. The relevant literature about its distinct pathologic features, clinical course and treatment is reviewed.
 ...
PMID:Langerhans' cell histiocytosis (histiocytosis-X) in a women with typical ultrastructure of Birbeck's granule: a case report. 899 34


1 2 3 4 5 6 7 8 9 10 Next >>