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Biomedical subjects

T Johnsen

Publications and source records attributed to T Johnsen.

At least 19 recordsLinked to original sources
PubMed1991

Temporal bone findings in a family with branchio-oto-renal syndrome (BOR).

A family group with confirmed branchio-oto-renal (BOR) syndrome was investigated in this study. Computerized tomography of the temporal bones has demonstrated that the malformations of the inner ear consist of hypoplastic structural changes within the cochlea with reduced vertical diameters, and absent or hypoplastic semicircular canals and normal endolymphatic ducts. It is concluded that in the present cases, the Mondini malformation of the cochlea is not associated with the BOR syndrome.

Adult↗
PubMed1989

CT-scanning of the cochlea in Pendred's syndrome.

The inner ears of 5 adult patients with Pendred's syndrome were investigated using a Siemens SOMATOM DRG. Five normal hearing adults participated in the investigation as a control group. The CT-scanning comprised 10-15 consecutive scans of the cochlea. The CT evaluation was performed using both a special bone setting and a soft tissue setting. The cochlear content was quantitatively evaluated by drawing an irregular region of interest on the bone pictures. The region of interest was then transferred to the soft tissue pictures by means of the standard program of the CT-scanner. The resulting mean values of attenuation expressed in Hounsfield Units were significantly lower in the Pendred cochleas than in the normal cochleas. At the same time a typical Mondini malformed cochlea was demonstrated in all patients with Pendred's syndrome. The lower values of attenuation of the cochlea in Pendred's syndrome reflect the rudimentarily developed infra-cochlear osseous structures in this disease. We conclude that CT-scanning of the cochlea using this procedure is reliable enough to replace the conventional axial-pyramidal tomography when a Mondini cochlea is suspected.

Adult↗
PubMed1989

The variable intrafamiliar expressivity in Pendred's syndrome.

This study presents clinical, audiological, radiological and biochemical data on 14 individuals representing 6 families, each including at least one patient with the classical Pendred's syndrome. The size of the thyroid, degree of hearing loss and result of the iodine perchlorate discharge test showed great variations, even between affected relatives. Based on these observations a new definition of Pendred's syndrome is proposed. The use of the iodine perchlorate discharge test for detection of healthy carriers among family members is suggested.

Adolescent↗
PubMed1988

Thyroid function in patients with Pendred's syndrome.

Thyroid function was studied in 17 unrelated patients with Pendred's syndrome. Fourteen patients had been treated with L-thyroxine, which was withdrawn during the investigation. Eight of the patients had previously had a thyroid resection. Thirteen patients had goiter at the time of study. The serum total thyroxine and serum total triiodothyronine concentrations were normal in 8, of whom 3 had elevated serum TSH concentrations. In the remaining 9 cases the thyroxine levels were below normal with elevated TSH. Serum reverse triiodothyronine concentrations were decreased in 8 out of 11. Median serum thyroglobulin was 973 micrograms/l (range 10.9-3200 micrograms/l) and increased in 13. Three patients had slightly positive thyroglobulin antibodies and one with normal level was thyrodectomized. Thyroid stimulating antibodies as measured by adenylate cyclase stimulation (median 114%, range 85-137%) were slightly increased in 11. When measured as TSH binding inhibiting immunoglobulins none were positive. Thyroid microsomal antibodies were negative in all. All patients with a detectable 131I uptake (n = 15) showed a pathological iodide perchlorate discharge test (median 32%, range 16-46%). These findings indicate an organification defect with impaired hormone synthesis.

Adolescent↗
PubMed1987

Pendred's syndrome. Acoustic, vestibular and radiological findings in 17 unrelated patients.

Seventeen unrelated Danish patients with Pendred's syndrome, whose case stories have not been published previously, are presented. Acoustic and vestibular functions were examined and endocrinological screening was performed. There was a great variation in hearing ability as well as in thyroid function. Furthermore, in contrast to previous investigations, normal caloric function was demonstrated in the majority. In all patients a Mondini malformation was demonstrated. On the basis of this investigation it is concluded that: (1) the Mondini defect is part of Pendred's syndrome; (2) the inherited Mondini malformation is the underlying cause of the sensorineural hearing impairment; and (3) the hearing sensitivity varies greatly in these patients.

Adolescent↗
PubMed1986

Temporal aspects of spatial adaptation. A study of the tilt aftereffect.

Growth and decay characteristics of the tilt aftereffect were studied for aftereffects induced by normal or continuous adaptation routines, and for aftereffects induced by successive or spaced adaptation to the same or different orientations on an adapt-partial decay-readapt schedule. In the continuous adaptation condition, growth and decay of the aftereffect were logarithmic functions of time. There was no evidence for saturation after 30 min adaptation. Aftereffect decay following spaced adaptation progresses as by continuous adaptation, but an adapting stimulus introduced during recovery from previous adaptation is more effective on the time scale than when introduced to a fully recovered system, summing approximately linearly with the residual aftereffect and off-setting the recovery process to zero. A second adapting stimulus whose orientation is of opposite sign (ccw vs cw) induces a two-phased decay process consisting of an early cancellation and a later enhancement of the original aftereffect. A two-stage model of adaptation is proposed.

Adaptation, Ocular↗
PubMed1986

Treatment of Graves' ophthalmopathy with cyclosporin A.

Six patients with Graves' ophthalmopathy (2 with acute and 4 with chronic alterations) were treated with cyclosporin A (10 mg/kg/day) for 5 weeks. This treatment had no effect on either the ocular manifestations (protrusion, eye muscle function) or subjective well-being of the patients. In contrast, creatinine clearance decreased from 83.5 to 55.5 ml/min during treatment, but normalized (94.9 ml/min) after cessation of the drug. A transient increase in serum 4-androstenedione was observed in 3 patients. We conclude that cyclosporin A has no convincing effect in the treatment of Graves' ophthalmopathy, but rather exerts serious renal effects.

Adult↗
PubMed1985

Visual half-field symmetry in orientation perception.

The perception of orientation in the left and right visual half-fields has been investigated. No evidence for interfield differences was obtained for the discrimination of single lines by line matching or in magnitude of the systematic orientation distortion in orientation contrast and rod-and-frame experiments. Furthermore, increasing the time interval between test and comparison lines in successive matching provides no evidence for a differential operation of short-term spatial memory in the two hemispheres. It is concluded that hemispheric asymmetries do not arise at the level of sensory processing of spatial signals.

Female↗
PubMed1985

Skeletal muscle in paramyotonia congenita: biochemistry, histochemistry and morphology.

In 12 patients with paramyotonia congenita, percutaneous needle biopsies from the brachial biceps muscle were performed. Muscle fibre area, distribution of muscle fibre types I, II-A and II-B and capillarization were not different from healthy controls. Signs of myopathy with central nuclei in the muscle cells were noted in 9 of the patients. 4 of these patients also had small areas with degeneration and, in one, vacuoles were observed. Quantitative determination of muscle glycogen, water and protein content were within normal range as were enzyme activities for hexokinase, lactate dehydrogenase, citrate synthetase and 3-hydroxy-acyl-CoA dehydrogenase.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1984

Zimeldine versus nomifensine. A double-blind study of depressed inpatients.

A double-blind comparison of zimeldine, a selective 5-HT reuptake inhibitor, and nomifensine, a noradrenaline and a dopamine reuptake inhibitor, was carried out in 43 inpatients with a scheduled treatment period of 6 weeks. All patients were diagnosed as definite major depressive disorder according to Research Diagnostic Criteria (RDC), and the WHO International Classification of Diseases (ICD-9). The antidepressive efficacy was evaluated by a 10-item subscale of the Comprehensive Psychopathological Rating Scale (CPRS), a clinical global impression (CGI) scale and a self-rating scale (VAMS). Side effects were recorded, and anticholinergic effect was evaluated by parotid saliva volume measurement. No statistically significant differences in efficacy or profile between the two drugs were demonstrable. With the exception of increased sweating in the zimeldine group there were no statistically significant differences in side effects.

Bipolar Disorder↗
PubMed1983

Effect of zendium toothpaste on recurrent aphthous stomatitis.

A double-blind clinical trial with cross-over was conducted for a period of 12 months in 25 patients with recurrent aphthous stomatitis (RAS). The effect of the amyloglucosidase and glucoseoxidase containing Zendium toothpaste on the discomfort, number of exacerbations, duration of exacerbation, number of ulcers and number of days with pain caused by RAS was studied. The use of Zendium significantly reduced the sensation of discomfort from RAS as compared to the use of placebo toothpaste (0.025 greater than p greater than 0.01). However, the patients were unable to discriminate significantly between Zendium and placebo when asked to choose one of the toothpastes (0.10 greater than p greater than 0.05) and no significant differences were demonstrated as far as the above mentioned parameters of disease severity were concerned. Therefore, it is concluded that the reducing effect of Zendium containing amyloglucosidase and glucoseoxidase on RAS is weak as compared to a similar toothpaste without these enzymes.

Adolescent↗
PubMed1979

Insulin receptors, insulin secretion, and glucose disappearance rate in patients with periodic hypokalaemic paralysis.

In a study of 6 male patients with periodic hypokalaemic paralysis (PHP), we found reduced insulin binding to monocytes as compared with a group of 25 normal subjects (P less than 0.1). The decreased insulin binding was caused by the decreased binding affinity. During induction of paralysis by a prolonged oral glucose load, one patient showed 24-h variations in the insulin binding to monocytes not differing from those observed in normals. After iv administration of glucose, these patients showed an elevated initial insulin response compared with the normals (P less than 0.1). However, the iv glucose tolerance tests revealed normal glucose disappearance rates. We conclude that changes in insulin receptor binding do not appear to be of pathophysiological significance for eliciting the parese attacks in PHP. However, the increased insulin response, following carbohydrate intake, might be of significance in the generation of paralytic attacks in patients with PHP.

Adolescent↗
PubMed1978

Skeletal muscle characteristics and carbohydrate metabolism after glucose loading in hypokalaemic periodic paralysis.

A prolonged glucose load was administered to four patients with hypokalaemic periodic paralysis and four healthy control sujbects. Muscle ATP and CP concentrations as well as lactate dehydrogenase, hexokinase and phosphorylase activities were similar in those two groups, but succinate dehydrogenase was approximately 50% higher in the control muscles. Muscles fibre composition was almost identical in the two groups, whereas patients had a higher degree of capillarization. Complete muscle weakness was produced in all patients, accompanied by hypokalaemia. Glucose loading resulted in elevated insulin levels and a minor rise in blood glucose level was seen in the patients compared to the control subjects. Glucose loading decreased hexokinase activity in controls, but increased this in the patients. At similar times, muscle and blood lactate levels and blood pyruvate values were generally higher in the patients over the course of the experiment. Initial glycogen concentrations were higher in patients, but glucose loading did not result in greatly increased glycogen values. These data suggest that patients with hypokalaemic periodic paralysis have an enhanced metabolism of carbohydrates and that insulin seems to be an important factor leading to the onset of muscle weakness.

Adenosine Triphosphate↗
PubMed1977

Endogenous insulin fluctuations during glucose-induced paralysis in patients with familial periodic hypokalemia.

Endogenous insulin production in patients with familial periodic hypokalemia has not previously been studied during induced attacks. The serum insulin, serum potassium, and blood glucose concentrations were measured in six patients with familial periodic hypokalemia during six attacks of paralysis induced by long-lasting glucose stimulation. The same parameters were measured in four normal subjects under the same conditions. There was no difference in insulin response or in blood glucose between the two groups. Basal insulin levels showed no difference. There was no correlation between the occurrence of the attack and the serum insulin level in the patients. All the patients responded by severe paralysis and hypokalemia.

Adult↗