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)

An asymmetric distribution of free fatty acids (FFA) is shown to occur between right and left cerebral hemispheres (RCH, LCH) of the rat. The RCH contains 35% less FFA than the LCH, the difference being mainly accounted for by saturated and monoenoic fatty acids. Acute and chronic electroconvulsive shock (ECS) affects the distribution and apparent rate of fatty acid production differently in each hemisphere. Taking into consideration the basal content of each hemisphere, RCH produces significantly higher amounts of FFA during the acute tonic phase of the convulsion evoked by a single ECS. The largest increases correspond to arachidonic and stearic acids (1800% and 420% in RCH, 1200% and 330% in LCH, respectively). The hemispheric sidedness is evened out after successive ECSs. The removal of the released fatty acids is also faster in the RCH, as suggested by its lower FFA levels 5 min after a single shock (the acute condition) or after the last of a series of 5 daily shocks (the chronic condition). The endogenous FFA content and composition is altered by chronic ECSs. Thus, 24 h after the last of a series of 4 daily ECSs, total FFAs remain about 40% higher than in the controls for both hemispheres. Arachidonic acid increase amounts to 150%, doubling its percentage contribution to the FFA pool. The lower endogenous FFA content in RCH, its higher responsiveness to ECS, and its ability to more rapidly recover the pre-convulsive levels, suggest that the deacylation and reacylation of complex lipids are more active in this hemisphere.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Endogenous asymmetry of rat brain lipids and dominance of the right cerebral hemisphere in free fatty acid response to electroconvulsive shock. 402 29

A retrospective analysis of 40 cases with histiocytosis X was undertaken to find out the course of primarily localized disease, and the prognosis of children with initially disseminated disease. Bone lesions recurred in nine of 23 children with localized histiocytosis X. In eleven cases other organ manifestations occurred as well; in four cases without bone relapse. After an observation period of 1-14 3/12 years, nine of 22 children in remission suffer from long-term sequelae like diabetes insipidus, convulsion, extrahypothalamic CNS-disease, orthopedic disability, growth retardation, dystrophia adiposogenitalis , and chronic headache. Four of 17 children with disseminated histiocytosis X died. Our results and others from the literature indicate various risk factors to be prognostically significant. 1) age less than 2 years 2) involvement of spleen and/or lung 3) elevated Lahey-score 4) dysfunction of the hematopoietic system, liver, and/or lung 5) histologic feature resembling malignant type 6) no response to therapy 7) severely affected general health. These factors can be evaluated initially. Considering our own experiences and some risk factors we suggest the definition of four risk groups: 1.) localized histiocytosis X of bone, lymph nodes or skin; 2.) disseminated histiocytosis X with benign histologic type and Lahey-score of one or two; 3.) Lahey-score of 3-8; 4.) disseminated histiocytosis X with dysfunction of certain organ systems and/or malignant histology.
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PMID:[Histiocytosis X--a retrospective analysis of 40 cases with localized or disseminated disease]. 661 Jan 8

A 31-year-old overweight man, suffering from high-blood pressure, was hospitalized for transient fits and hemiparesis. MRI disclosed a large irregular mass affecting the vault, meninges and invading the parietal lobe. At neurosurgery, the lesion was necrotic, hemorrhagic and poorly demarcated from the surrounding brain. Histopathology revealed a benign Langerhans cell histiocytosis. No other systemic nor organic lesions could be discovered. After additional local radiotherapy, the patient recovered completely and regained normal activities 6 months later.
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PMID:Focal brain invasion as the first manifestation of Langerhans cell histiocytosis in an adult. Case report. 840 25

Among adults, liver involvement is relatively frequent in Langerhans' cell histiocytosis (LCH), even though it is often overlooked. In fact, the liver involvement may be missed in apparently localized LCH or when it is the sole site of involvement. We present 23 cases of liver involvement in LCH out of a cohort study of 85 adult patients included in the French Histiocytosis Study Group Registry. The most frequent clinical setting was multiorgan involvement (87% of our cases). The main histological pattern in liver LCH was sclerosing cholangitis (56% of the cases). The symptoms included hepatomegaly (48%) and/or liver biochemistry abnormalities (61%, including cholestasis associated with increased transaminases levels in 35% of cases, cholestasis only in 22% and increased transaminases levels only in 4% of the cases). Particularly suggestive of the diagnosis was the observation of biliary tree abnormalities through magnetic resonance imaging (MRI). The natural history of liver LCH fits into two stages: early infiltration by histiocytes and late sclerosis of the biliary tree. We found that liver involvement had a significant impact on survival. Thus we suggest that clinical and biological liver evaluation must be performed regularly onwards to screen every LCH adult patient from the time of the initial diagnosis. MRI and liver biopsy should be considered as soon as the data point to a possible liver localization. If this diagnosis is confirmed, we suggest a treatment with ursodesoxycholic acid, as in other cholestatic diseases, together with treatments specifically directed towards LCH. However, the ideal treatment of liver LCH remains to be found, and in advanced cases transplantation is the sole option.
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PMID:Langerhans' cell histiocytosis of the liver in adults. 2155 Mar 30

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.
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PMID:Adult langerhans cell histiocytosis with hepatic and pulmonary involvement. 2597 28