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  • 1
    ISSN: 1432-0428
    Keywords: Insulin delivery rate ; insulin secretion in man ; glucose ; tolbutamide ; insulin disappearance rate ; biphasic insulin secretion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Serum insulin response to a single bolus of IV glucose or tolbutamide was measured in eight healthy subjects. Insulin disappearance rate was assessed by deconvolution from the serum insulin levels, using the measured insulin disappearance rate. The mean rate constant of insulin disappearance was 0.238±0.005 min−1 (mean±SEM). Basal insulin delivery rate was 8.0 to 9.0 mU/min and the delivery rate following glucose injection (0.5 g/kg body weight) showed a biphasic response, whereas that after tolbutamide injection (15.6 mg/kg body weight), a monophasic response. After glucose injection, 1.7±0.3 U of insulin was delivered during the first phase (0–10 min) and 5.6±1.6 U during the second phase (11–60 min). After tolbutamide injection, 1.5±0.3 U of insulin was delivered during the first 10 min. Between 11 and 40 min, 1.6±0.5 U of insulin was delivered. The results thus confirm and also quantitate biphasic insulin secretion after a bolus of glucose with a monophasic response after tolbutamide. The method is suitable for studies of the insulin secretogogues in man.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 76 (1988), S. 208-211 
    ISSN: 1432-0533
    Keywords: Familial amyotrophic lateral sclerosis ; Sporadic lower motor neuron disease ; Lewy body-like inclusions ; Bunina bodies ; Cord-like thickenings of cell processes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A sporadic case of lower motor neuron disease with Lewy body-like inclusions is presented. A woman of 69, 6 months before death, had a history of muscle weakness and atrophy of the four extremities. Neuropathological examination revealed neuronal loss and associated gliosis in the anterior horns of the whole spinal cord. The cytoplasm of some of the remaining neurons contained Lewy body-like inclusions, Bunina bodies, or both. Spheroids and cordlike thickenings of cell processes were also observed in the anterior horns. A few neurons showed argentophilia of the cytoplasm. No tract degeneration was detectable in the white matter of the spinal cord. The present case and two reported similar cases may constitute a new subgroup of motor neuron disease.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Senile dementia of Alzheimer's type ; Alzheimer's disease ; Pick's disease ; Laminar neuronal loss ; Limbic system
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Seven cases of senile dementia of Alzheimer type (SDAT) with unusual clinico-pathological findings are reported. The patients showed neuronal loss in laminar pattern, with gliosis exclusively confined to the CA1 of the hippocampus, the area of the hippocampal gyrus (entorhinal cortex) and medial occipitotemporal cortex. This change was more pronounced in the oral region. The subcortical white matter showed more pronounced fibrillary gliosis than loss of myelin. Both Alzheimer's neurofibrillary tangles and senile plaques were less marked than those usually seen in SDAT. The mental disturbance started after the age of 65 in all patients. The main clinical feature was marked character change in addition to disturbance of cognitive function. Cranial computed tomography showed marked dilatation of the oral portion of the inferior horn of the lateral ventricle in the early stage. It was apparent that although the cases in this group could be incorporated within in the spectrum of SDAT, they could also be considered to represent a variant of SDAT. This group could contribute to an understanding of the clinico-pathological spectrum of SDAT as well as indicating ways of managing such patients.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Calcitonin gene-related peptide ; Motor neuron disease ; Neurofilament ; Spheroid ; Spinal cord
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The spinal cords from seven autopsy cases of sporadic motor neuron disease (MND) and two controls were immunohistochemically examined using anti-bodies directed to calcitonin gene-related peptide (CGRP) and to neurofilament proteins (Nf). CGRP immunoreactivity was observed in the posterior horns, especially in the laminae I and II, of all the spinal cords examined. In MND cases in addition, a considerable number of spheroids in the anterior horns were labelled with the antibody. In some spheroids, their entire area was homogeneously immunostained, whereas in others the immunoreactivity was confined to a focal area(s) within the profile of the spheroids and between these two forms of staining several variations of the staining patterns were seen. The anti-Nf intensely and homogeneously labelled all spheroids and there was no appreciable difference in the Nf-immunoreactive pattern, between CGRP-posittive and-negative spheroids. It is possible that the accumulation of CGRP in spheroids may result from entrapment of CGRP during the anterograde axonal transport causing loss of CGRP at the neuromuscular junction and producing weakness and atrophy of the muscles.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 77 (1989), S. 391-396 
    ISSN: 1432-0533
    Keywords: Bunina body ; Hyaline inclusion body ; Lewy body like inclusion ; Sporadic motor neuron disease ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Round eosinophilic hyaline inclusion bodies with halos in the somata of anterior horn cells from a case of sporadic lower motor neuron disease (MND) were intensely immunostained with the monoclonal anti-ubiquitin antibody (DF2). A few similar, DF2-positive inclusions were also observed in the nerve cell processes of anterior horn cells or in the neuropil. Most inclusions showed intense homogeneous staining of the entire inclusion, whereas a few had intense staining of their periphery with no or pale staining of the central areas. Other DF2-positive structures in the somata of anterior horn cells included cytoplasmic granular structures, eosinophilic thread-like or reticular structures, and small eosinophilic profiles different from Bunina bodies. The DF2-staining intensity of Bunina bodies and spheroids did not exceed the background level. These results suggest that ubiquitination is associated with a pathological process of anterior horn cell degeneration in this MND case.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1238
    Keywords: Inferior vena cava pressure ; Arterial ketone body ratio ; Hepatic congestion ; Liver viability ; Fontan operation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Objective Abnormally elevated central venous pressure is considered to be an etiological factor in the onset of acute hepatic failure following modified Fontan operation. This paper hypothesises that an increase in inferior vena cava pressure (IVCP) after such an operation has adverse effects on hepatic energy status. Design Various degrees of venous hypertension were produced in 10 mongrel dogs by clamping the thoracic IVC with an active veno-venous shunt and varying its flow rate from 60–2.5 ml/min/kg. Arterial ketone body ratio (KBR), reflecting the hepatic mitochondrial redox state, was measured as an index of hepatic energy status.Measurements and results: The lower the flow rates of the shunt, the higher the pressures of IVC and portal vein, while systolic blood pressure was maintained above 100 mmHg. CO significantly decreased when the pump speed was less than 30 ml/min/kg. KBR showed a negative correlation to IVCP as well as a positive correlation to portal blood flow (p〈0.05). Conclusion From the simple regression line obtained between IVCP and KBR, it was determined that an upper safety limit of IVCP may lie at about 27 cmH2O (20.5 mmHg), and that a IVCP of 35 cmH2O (26.6 mmHg) seems to be the critical level for maintaining liver viability.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 135 (1980), S. 69-71 
    ISSN: 1432-1076
    Keywords: Phenytoin toxicity ; Michaelis-Menten kinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two girls aged 6 and 10 years treated with anticonvulsants developed nystagmus and ataxia. The peak plasma concentrations of phenytoin were 42.6 and 46.6 μg/ml, respectively, compatible with phenytoin toxicity. The decline in plasma phenytoin levels did not fit first order kinetics, but followed Michaelis-Menten kinetics.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Magnetic resonance imaging ; Growth hormone deficiency ; Head trauma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two patients are reported with growth hormone deficiency due to head trauma in childhood. Although their injuries were outwardly only slight and there was no loss of consciousness and no subsequent neurological deficits, they exhibited gradual growth retardation from the time of the trauma. Provocative endocrinological tests showed growth hormone deficiency and MRI showed transection of the pituitary stalk. These findings suggest that ordinary head trauma, as well as perinatal insult and congenital abnormalities, could be a cause of growth hormone deficiency.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Serum bile acids ; Alagille syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To clarify whether an abnormal bile acid pattern has a role in the pathogenesis of Alagille syndrome, we compared serum bile acid patterns in seven with Alagille syndrome with those of patients with congenital biliary atresia (CBA), neonatal hepatitis (NH) and normal infants. Of the seven patients with Alagille syndrome, four patients were younger and three were older than 1 year. The mean total serum bile acid level in the infants was higher than in older subjects. There was a dissociation between the levels of serum total bile acid and bilirubin in three of the seven cases. The mean total bile acid levels in serum were in the following decreasing order: CBA, Alagille syndrome, NH and controls. The ratio of cholate to chenodeoxycholate in the younger patients with Alagille syndrome was significantly higher than CBA (P〈0.001). However, no specific bile acid pattern was found in Alagille syndrome by high-performance liquid chromatography (HPLC).
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 151 (1992), S. 321-325 
    ISSN: 1432-1076
    Keywords: Short stature ; Somatomedin-C ; IGF-I ; IGF-I resistance
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a Japanese girl with short stature, malar hypoplasia, up-slanting palpebral fissures, blue sclerae and thin, stiff and slightly brownish hair. Short stature started in utero and her psychomotor development was normal. Menarche appeared at 13 years 8 months. Height at 14 years 5 months was 132 cm (−4.6 SD). Her growth hormone (GH) sleep pattern and responses to insulin,l-dopa, arginine, propranolol-glucagon and growth hormone-releasing hormone were normal. Plasma insulin-like growth factor I (IGF-I) was high (2170–4860 units/l) and increased from 4860 to 7080 units/l 20 h after biosynthetic GH injection. Gel infiltration patterns of the free and protein-bound IGF-I in plasma from the patient were not different from the controls; IGF-I fraction of the high and low molecular weight binding protein and the non-protein bound fraction were 75.5%, 15.8% and 8.7%, respectively. IGF-I from the patient showed normal bioactivities when determined by [35S]sulphate and [3H]thymidine uptake into cultured rat chondrocytes, and by [3H]thymidine and [3H]α-aminoisobutyric acid uptake into the patient's skin fibroblasts. IGF-I binding to cultured skin fibroblasts from the patient was comparable to that of controls. These results suggest that tissue specific defects of IGF-I receptors may be the cause of increased IGF-I levels in the patient.
    Type of Medium: Electronic Resource
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