ZIB

Hits per page

hits 1 - 2 | 2 hits

Sorting

Electronic Resource

Type I Gaucher disease: extraosseous extension of skeletal disease (2000)

Poll, L. W. ; Koch, J. -A. ; vom Dahl, S. ; [et al.]

Springer

Skeletal radiology 29 (2000), S. 15-21

add to mindlist on the mindlist

Details

ISSN: 1432-2161

Keywords: Key words Gaucher disease ; Bone disease ; Extraosseous Gaucher disease ; Bone marrow imaging ; MRI

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: Abstract Objective. To investigate the frequency and morphology of extraosseous extension in patients with Gaucher disease type I. Design and patients. MRI examinations of the lower extremities were analyzed in 70 patients with Gaucher disease type I. Additionally, the thoracic spine and the midface were investigated on MRI in two patients. Results. Four cases are presented in which patients with Gaucher disease type I and severe skeletal involvement developed destruction or protrusion of the cortex with extraosseous extension into soft tissues. In one patient, Gaucher cell deposits destroyed the cortex of the mandible and extended into the masseter muscle. In the second patient, multiple paravertebral masses with localized destruction of the cortex were apparent in the thoracic spine. In the third and fourth patient, cortical destruction with extraosseous tissue extending into soft tissues was seen in the lower limbs. Conclusions. Extraosseous extension is a rare manifestation of Gaucher bone disease. While an increased risk of cancer, especially hematopoietic in origin, is known in patients with Gaucher disease, these extraosseous benign manifestations that may mimic malignant processes should be considered in the differential diagnosis of extraosseous extension into soft tissues. A narrow neck of tissue was apparent in all cases connecting bone and extraosseous extensions.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/s002560050003

Permalink

Library	Location	Call Number	Volume/Issue/Year	Availability

Others were also interested in ...

Paper (German National Licenses)

Fulltext

Electronic Resource

Exposure of perfused liver to hypotonic conditions modifies cellular nitrogen metabolism (1990)

Häussinger, D. ; Lang, F.

New York, N.Y. : Wiley-Blackwell

Journal of Cellular Biochemistry 43 (1990), S. 355-361

add to mindlist on the mindlist

Details

ISSN: 0730-2312

Keywords: cell volume ; glutamine ; ammonia ; urea synthesis ; metabolic regulation ; Life and Medical Sciences ; Cell & Developmental Biology

Source: Wiley InterScience Backfile Collection 1832-2000

Topics: Biology , Chemistry and Pharmacology , Medicine

Notes: Isolated livers were exposed to hypotonic perfusates. As shown previously, this hypotonic challenge leads to initial cell swelling, followed by volume regulatory ion fluxes, largely restoring cell volume within approximately 6 min. However, the hepatocyte is left in an altered metabolic state, which is characterized by marked stimulation of hepatic glutamine uptake and degradation and transient release of glutamate from the liver. Urea formation from glutamine and alanine is stimulated, whereas hepatic ammonia uptake and utilization for urea and glutamine synthesis decreases. These observations reveal a hitherto unrecognized factor modulating hepatic function during intestinal absorption.

Additional Material: 3 Ill.