Primary myelodysplastic syndrome: treatment of 6 patients with 13-cis retinoic acid.
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Biomedical subjects
Publications and source records attributed to B Pedersen.
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Relations between cytogenetic status, FAB-classification and an abnormal subpopulation of myeloperoxidase (MPO)-deficient polymorphonuclears (PMN) in 45 patients with myelodysplastic syndrome (MDS) are reported. Clonal abnormalities were demonstrated in 85% of the patients, with a lower incidence in the RA+ group (refractory anaemia with ring sideroblasts) compared to the others (p = 0.004). In 12 patients a spontaneous progression in cytogenetic aberrations occurred and in 7 of these (60%) a simultaneous progression in FAB-subtype was seen. The appearance of MPO-deficient PMNs was observed in 6 of these patients (55%). A progression in FAB-subtype was noted in further 4 patients and 2 additional patients developed MPO-deficient PMNs. Only one sufficient cytogenetic investigation was available in these patients. Thus 100% of the fully studied patients showed progression in cytogenetic abnormalities when a progression in FAB-subtype or a development of MPO-deficient PMNs was seen. 3 (49%) of the 8 patients developing MPO-deficient PMNs too showed a progression in FAB-subtype. Although no significant correlation to specific categories of structural aberrations or abnormalities in specific chromosome pairs could be demonstrated, clonal cytogenetic aberrations seem to be involved when the disease progresses and when MPO-deficient PMN develop.
The ganglion Gasseri and the brainstem were examined in three old patients with herpes zoster without predisposing diseases with special reference to the mesencephalic trigeminal nucleus. The primary lesions in the semilunar ganglion vary with the length of the course. Secondary changes in the brainstem were as expected from pons to the second cervical segment of the cord. Besides, we observed degeneration, inflammation and glial nodules in the mesencephalic trigeminal nucleus in two patients. According to Brodal this nucleus presumably corresponds to the semilunar and spinal ganglia. As herpes zoster virus is prone to attack the sensory nuclei our findings support this hypothesis.
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We review dystonia treatment results since 1981, including our own findings. Anticholinergics are still the most effective drugs, but less than 50% of patients continue with treatment. The authors recommend a combination of an anticholinergic, a benzodiazepine, and another drug (an antidopaminergic, carbamazepine, or fluperlapine) for the treatment of dystonia.
The serum-valproate level of four patients with epilepsy was followed during pregnancy. A decrease in serum level occurred late in pregnancy and was followed by a pronounced increase in the first week after delivery. The maternal serum concentration of valproate was compared to that of the umbilical cord. The level in cord blood was 145-219% higher than that in maternal blood. The concentration of valproate in breast milk was found to be 5-10% of the maternal serum concentration. The serum concentration was measured in one breastfed child. The level was 7.6% of the maternal serum concentration. All children were healthy without any signs of intoxication or malformation. Based on our experience, pregnant patients treated with valproate must be carefully controlled especially during the last month of pregnancy and in the first two weeks after delivery. The amount of valproate excreted into the breast milk was negligible and should not prevent breast feeding.
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Previous herpes simplex virus type 2 (HSV-2) infections are known to predispose women for the development of cervical cancer, but causal relationship between the virus and the cancer has never been proven. Forty-six patients with cervical carcinoma (13 with preinvasive lesions and 33 with various stages of invasive disease) were selected for the present study. Among the patients 96% were seropositive for the presence of antibodies to herpes simplex virus compared to 87% of the 30 controls. Antibodies specific for HSV-2 were found in the sera from 24% of the patients and 17% of the controls by the use of an immunoblotting test. Patients and controls were typed for HLA-A,B,C and D/DR antigens, but no significant associations were found.
Bone marrow from a patient with chronic myeloid leukemia in blastic phase at diagnosis showed cells with t(9;22), as well as others with a 22q+ marker. The marker was due to t(8;22), and consequently, these cells presented a masked Ph1: t(8;9;22) (q24.2;q34;q11). The marrow contained a wide variety of abnormal clones, which formed an evolutionary pedigree. The pedigree allowed location of the stage of evolution at which masking of the Ph1 had taken place.
A case of severe valproic acid poisoning with coma and insufficient respiration is reported. The clinical condition and EEG changes are presented. The patient recovered completely, and the toxicity of sodium valproate to the brain and the liver in severe intoxication is discussed.
Earmoulds according to the horn principles often lack the necessary space for a vent parallel to the sound canal. The present paper shows that a 2 mm vent ending in the sound canal itself near the tip of the earmould does not affect the frequency characteristics of the hearing aid measured at the eardrum.
Due to individual variations in the auricle, meatus, middle ear, and the ear mould, identical hearing aids will yield different insertion gain in different ears. The present experiment was performed in order to introduce an adjusting factor for the individual ear mould-supplied patient compared with KEMAR data as a basis for the selection and fitting of the hearing aid. For different hearing aids tested on the same person the variation in the insertion gain compared with KEMAR data was, however, of such an extent that such a factor cannot be immediately introduced. The variation reflects differences in the sensitivity of the hearing aids with respect to the acoustic load represented by the ear mould-fitted individual ear. In order to predict the effect of a certain hearing aid on one specific patient it is necessary to know the sensitivity for the acoustic load impedance in advance.
The literature has been reviewed for cases of malignant disease showing cellular clones with 24-36 chromosomes. Such cases are characterized by aggressive disease. Together the clones with 24-36 chromosomes compose a remarkably consistent non-random cytogenetic pattern, which demonstrates that different chromosomes are of different value for cellular survival and clonal propagation. Considering that the tissues of origin are very different (various solid tumours and different leukaemias), the close mutual cytogenetic relationship between the clones indicates that the cytogenetic pattern is tissue non-specific. Karyotypic non-specificity of cells with very different phenotypes is an apparent contradiction, which raises important questions concerning the relation between karyotype and phenotype.
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