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  • 1
    Electronic Resource
    Electronic Resource
    Gaucher disease of the spleen: CT and MR findings (2000)
    Springer
    Abdominal imaging 25 (2000), S. 286-289 
    Details
    ISSN: 1432-0509
    Keywords: Key words: Gaucher disease—Spleen in Gaucher disease—Splenic nodules—Computed tomography—Magnetic resonance imaging.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present a 26-year-old male patient with Gaucher disease who presented with epigastric pain and a palpable mass in the left abdomen. Ultrasound, abdominal computed tomography, and magnetic resonance imaging showed massive splenomegaly with multiple splenic nodules up to 7 cm in diameter. Splenic nodules should be included in the differential diagnosis of splenic masses. Follow-up is necessary because of the increased incidence of hematologic malignancies in Gaucher disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002610000010
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  • 2
    Electronic Resource
    Electronic Resource
    New clues to the pathophysiology of hepatorenal failure (1993)
    Springer
    Journal of molecular medicine 71 (1993), S. 93-97 
    Details
    ISSN: 1432-1440
    Keywords: Liver ; Kidney ; Nitroxide ; Substance P ; Hepatorenal reflex ; Glomerulopressin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In patients with advanced liver disease, decreases in renal blood flow, glomerular filtration rate, and urinary output are frequently observed. The deterioration in renal function is usually not due to a unique cause but is the result of the concerted action of several mechanisms operating in parallel; decreased plasma protein formation and increased intrahepatic vascular resistance lead to sequestration of blood volume, favoring hypovolemia and reduction in cardiac output. At the same time enhanced formation of nitroxide leads to peripheral vasodilation; bacterial endotoxin escaping clearance by the diseased liver stimulates the expression of a long-acting nitroxide synthase. Furthermore, vasodilating intestinal mediators such as substance P escape inactivation by the liver. In the face of peripheral vasodilation the maintenance of blood pressure requires an increase in cardiac output, which is achieved by activation of sympathetic nervous tone, renal vasoconstriction, enhanced release of renin, angiotensin, aldosterone, and antidiuretic hormone, leading to renal retention of sodium and water. Renal vasoconstriction is opposed by vasodilatatory prostaglandins, and renal failure may be triggered by inhibition of prostaglandin formation. On the other hand, vasoconstrictive eicosanoids, such as thromboxane B2 and leukotriene E2, which escape hepatic inactivation, may contribute to renal vasoconstriction. Beyond these mechanisms disturbed hepatic regulation of renal function may participate in the generation of hepatorenal syndrome. The liver regulates renal function via both a hepatorenal reflex decreasing renal blood flow and a hypothetical liver-borne diuretic factor increasing renal blood flow. Both enhanced hepatorenal reflex activity and decreased formation of the liver-borne diuretic factor could participate in the pathogenesis of hepatorenal syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00179987
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  • 3
    Electronic Resource
    Electronic Resource
    Central pontine myelinolysis: clinical presentation and radiologic findings (1996)
    Springer
    European radiology 6 (1996), S. 177-183 
    Details
    ISSN: 1432-1084
    Keywords: Myelin ; Brain stem ; Pons ; Magnetic resonance imaging ; Electrolyte disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Central pontine myelinolysis (CPM) is a neurologic disorder once thought to be uniformly fatal. With the introduction of CT and MRI there was an increasing number of reports on nonfatal cases of CPM. Nearly all reports on nonfatal cases describe severe clinical syndromes with tetraparesis, bulbar palsy, and coma. We reviewed nine patients with CPM and compared the size of the pontine lesion on MRI and CT with the severity of clinical presentation. Clinical presentation of CPM was highly variable: The symptoms ranged from severe neurologic disorders to mild neurologic disturbances only. Two of nine patients died from CPM. The size of the pontine lesion did not correlate with the severity of the neurologic illness or the final outcome. Mild forms of CPM might be difficult to diagnose clinically. This applies even more for patients with underlying diseases such as Wernicke's encephalopathy, which in itself might cause a clinical picture similar to that of CPM. Central Pontine Myelinolysis is a major differential diagnosis in acute neurologic deterioration indicating pontine damage. Magnetic resonance imaging is the decisive diagnostic tool for CPM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00181139
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  • 4
    Electronic Resource
    Electronic Resource
    Application of61Ni Mössbauer spectroscopy to chemical problems (1996)
    Springer
    Il nuovo cimento della Società Italiana di Fisica 18 (1996), S. 231-235 
    Details
    ISSN: 0392-6737
    Keywords: Mössbauer spectra ; Structure, bonding, conformation, configuration and isomerism of biomolecules ; Conference proceedings
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Summary Using the possibility to produce61Co sources at the MAinz MIcrotron,61Ni Mössbauer spectroscopy was applied to different kinds of chemical problems. Measurements of isomer shifts andV zz values in some common compounds and compounds with unusual chemical bonding for comparison with band structure calculations, measurement of61Ni Mössbauer parameters in model compounds for hydrogenase and dehydrogenase with an active [NiS4] centre, and study of novel binary NiF3 compounds at helium temperature are presented.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02458896
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  • 5
    Electronic Resource
    Electronic Resource
    Topical benzocaine anaesthesia lacks analgesic effects in painful non-acid oesophagitis (1997)
    Oxford, UK : Blackwell Science Ltd
    Alimentary pharmacology & therapeutics 11 (1997), S. 0 
    Details
    ISSN: 1365-2036
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: No established treatment exists for pain relief in symptomatic non-acid oesophagitis. One of the most common topical anaesthetics is benzocaine which has been demonstrated to produce excellent analgesia on oral mucous membranes.〈section xml:id="abs1-2"〉〈title type="main"〉MethodsIn a prospective, placebo-controlled, balanced, single-blinded study, 26 patients with retrosternal discomfort or odynophagia due to painful non-acid oesophagitis were treated either with oral benzocaine 0.75% solution (n = 14) or with benzocaine-free solvent (placebo, n = 12) at a daily dose of 20–40 mL, for up to 6 consecutive days (median 5.5 days). During the study period patients recorded subjective pain scores for both complaints on visual analogue scales.〈section xml:id="abs1-3"〉〈title type="main"〉ResultsBenzocaine did not affect pain scores for any of the two symptoms, nor did it alter global subjective or objective assessment of therapy outcome in treated compared to untreated subjects (P 〉 0.05). There was a non-significant tendency for placebo patients to stop prematurely their study medication more often, because of lack of analgesic efficacy (P = 0.098).〈section xml:id="abs1-4"〉〈title type="main"〉ConclusionsTopical benzocaine cannot be recommended for routine symptomatic pain relief in non-acid oesophagitis. By indirect evidence, it is assumed that pain perception of non-acid oesophagitis is not preferentially mediated by superficially located mucosal nociceptors.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2036.1997.00213.x
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  • 6
    Electronic Resource
    Electronic Resource
    Decrease of plasma taurine in Gaucher disease and its sustained correction during enzyme replacement therapy (2000)
    Springer
    Amino acids 19 (2000), S. 585-592 
    Details
    ISSN: 1438-2199
    Keywords: Keywords: Amino acids – Enzyme replacement therapy – Lysosomal storage disease – Macrophages – Liver disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary. Gaucher disease is caused by an autosomal-recessive deficiency of glucocerebrosidase. Cells of monocytic/macrophagic origin accumulate glucosylceramide. This leads to hepatosplenomegaly, bone destruction, thrombocytopenia and anemia. Enzyme replacement therapy (ERT) with macrophage-targeted glucocerebrosidase leads to normalization of these parameters. The way of macrophage activation in Gaucher disease is not known. Recently, the osmolytes taurine, betaine and inositol were identified as important regulators of macrophage function in liver. Therefore, the role of plasma taurine in Gaucher disease as a primarily macrophage-derived disease was studied. Fasting plasma levels were measured from blood samples of healthy control subjects (n = 29, m : f = 11 : 18, mean age 37 ± 3 years), from un-treated Gaucher patients (n = 16, m : f = 7 : 9, mean age 44 ± 4 years) and those treated for 37 ± 2 months (n = 54, m : f = 19 : 35, mean age 47 ± 2 years). Amino acid analysis was carried out in a BioChrom amino acid analyzer. In the untreated patients, plasma taurine was 45 ± 3 μM, as compared to the controls with a plasma taurine of 63 ± 4 μM (p 〈 0.01). The aver-age increase of plasma taurine during the first year of ERT was 18 ± 8 μM (n = 10). Patients treated for an average of 37 months (range 1–9 years of ERT) had a plasma taurine of 65 ± 4 μM (n = 54), which was not different from the controls. It is concluded that Gaucher patients show decreased plasma taurine levels and that therapy of Gaucher disease might correct this. It has to be established, whether decreased taurine availability is a cofactor of the permanent activation of glucosylceramide-storing monocytes/macrophages in this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s007260070008
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  • 7
    Electronic Resource
    Electronic Resource
    Extraosseous manifestation of Gaucher's disease type I: MR and histological appearance (2000)
    Springer
    European radiology 10 (2000), S. 1660-1663 
    Details
    ISSN: 1432-1084
    Keywords: Key words: Gaucher's disease type I – Skeletal disease – Bone marrow imaging – MRI
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Gaucher's disease type I is the most prevalent lysosomal storage disorder caused by an autosomal-recessive inherited deficiency of glucocerebrosidase activity with secondary accumulation of glucocerebrosides within the lysosomes of macrophages. The storage disorder produces a multisystem disease characterized by progressive visceral enlargement and gradual replacement of bone marrow with lipid-laden macrophages. Skeletal disease is a major source of disability in Gaucher's disease. Extraosseous extension of Gaucher cells is an extremely rare manifestation of skeletal Gaucher's disease. This is a report on the MRI and histopathological findings of an extraosseous Gaucher-cell extension into the midface in a patient with Gaucher's disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003300000446
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  • 8
    Electronic Resource
    Electronic Resource
    Regulation of ion channels in rat hepatocytes (1997)
    Springer
    Pflügers Archiv 435 (1997), S. 203-210 
    Details
    ISSN: 1432-2013
    Keywords: Key words Patch clamp ; Cell membrane potential ; K+ conductance ; K+ channels ; Cl ; channels ; cAMP ; Cell volume ; Progesterone ; Hepatocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Patch-clamp studies have been performed to elucidate single ion channels in rat hepatocytes. In rat hepatocytes two types of ion channel have been identified: an inwardly rectifying K+ channel with a mean inward conductance of 55 ± 6.5 pS (n = 20) and a mean outward conductance of 25 ± 3.2 pS (n = 20) in the inside-out configuration with 145 mmol/l KCl on either side of the patch as well as an outwardly rectifying Cl– channel with a mean outward conductance of 30 ± 4.5 pS (n = 8) and a mean inward conductance of 10 ± 2.3 pS (n = 6) in the inside-out configuration with symmetrical 145 mmol/l KCl. The open probability of these channels is virtually insensitive to Ca2+ activity on the intracellular side. Accordingly, the Ca2+ ionophore ionomycin had no effect on cell membrane potential. Dibutyryl-cAMP (db-cAMP) hyperpolarizes the cell membrane and increases the activity of the 55-pS inwardly rectifying K+ channel by reducing the duration of closure between bursts. Forskolin similarly hyperpolarizes the cell membrane. The inwardly rectifying K+ channel is inhibited by progesterone, while the outwardly rectifying Cl– channel is insensitive to progesterone.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004240050502
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  • 9
    Electronic Resource
    Electronic Resource
    Altered cell volume regulation in ras oncogene expressing NIH fibroblasts (1992)
    Springer
    Pflügers Archiv 420 (1992), S. 424-427 
    Details
    ISSN: 1432-2013
    Keywords: Cell volume regulation ; Ras oncogene ; Na+/H+ Exchanger ; Na+, K+,2Cl− Cotransporter ; Dimethylamiloride ; Furosemide ; Quinidine ; Bumetanide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Expression of the Ha-ras oncogene has been reported to stimulate the dimethylamiloride sensitive Na+/H+ exchanger and Na+, K+, 2Cl− cotransport, both transport systems which are involved in cell volume regulation. The present study has been performed to test for an influence of ras oncogene expression on cell volume regulation in NIH 3T3 fibroblasts expressing the Ha-ras oncogene (+ ras). As controls served NIH 3T3 fibroblasts not expressing the ras oncogene (− ras). In isotonic extracellular fluid, the cell volume of + ras cells (2.70±0.08 pl) is significantly greater than the cell volume of −ras cells (2.04±0.10 pl). Both, + ras and − ras cells exhibit a regulatory cell volume increase in hypertonic extracellular fluid and a regulatory cell volume decrease in hypotonic extracellular fluid. The regulatory cell volume decrease is inhibited by 1 mmol/l quinidine and barium, the regulatory cell volume increase is inhibited in − ras and + ras cells by dimethyl-amiloride (100 μmol/l) and, only in + ras cells, by furosemide (100 μmol/l) and bumetanide (10 μmol/l). In conclusion, expression of the ras oncogene leads to a shift of the set point for cell volume regulation to greater cell volumes, which may contribute to the activation of the Na+/H+ exchanger and Na+, K+, 2Cl− cotransport.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00374615
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  • 10
    Electronic Resource
    Electronic Resource
    Serotoninergic hepatorenal reflex regulating renal glomerular filtration rate (1991)
    Springer
    Pflügers Archiv 419 (1991), S. 111-113 
    Details
    ISSN: 1432-2013
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Infusion of glutamine (2 μmol/min) into the superior mesenteric vein leads to a decrease of renal glomerular filtration rate (GFR) and urinary flow rate (V), whereas infusion of identical amounts of glutamine into the jugular vein does not significantly alter GFR or V. The effect of mesenteric glutamine is mimicked by mesenteric infusion of 5 nmol/min serotonin and is abolished in the presence of 20 nmol/min methysergide. The effect of mesenteric serotonin is almost abolished after transection of vagal hepatic nerves. The observations point to a serotoninergic hepatorenal reflex regulating renal function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00373755
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